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J. Nielsen, K. Johansen and H. Yde


Glucose tolerance, plasma insulin and growth hormone response to a glucose load were studied in 10 patients with Turner's syndrome and 3 patients with pure gonadal dysgenesis. It was found that 60 per cent of the patients with Turner's syndrome had a diabetic GTT. This is a frequency which is significantly higher than expected. None of the patients with pure gonadal dysgenesis had a diabetic GTT.

The diabetes in patients with Turner's syndrome was of a mild type as is most frequently found in maturity-onset diabetes. The insulin response was, however, different from what is usually found in maturity-onset diabetes. The patients with Turner's syndrome showed a brisk rise and prolonged high levels of plasma insulin after glucose ingestion. An early plasma growth hormone peak was found in patients with Turner's syndrome. The significance of this finding for the pathogenesis of mild diabetes in patients with Turner's syndrome is discussed together with other possible aetiologic and pathogenetic factors.

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M. Fraccaro, D. Ikkos, J. Lindsten, R. Luft and K. G. Tillinger


A case of so-called male Turner's syndrome is reported in a 25 year old patient. Chromosomal studies revealed a normal male karyotype. The sex chromatin pattern was negative. Histological examination of the bilaterally cryptorchid testes showed a picture typical for intraabdominal testes.

Analysis of the published cases of so-called male Turner's syndrome revealed uniform histological changes of the testis in the form of slight to moderate alterations of the tubular walls and absence or immaturity of the cells of the germinal epithelium. The clinical, histological and hormonal findings in this syndrome have been tentatively defined.

The term testicular germinal dysgenesis is suggested as more appropriate for this syndrome than the commonly used name of male Turner's syndrome.

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Hans H. Bassøe and Hans-Kr. Krogh

One of the authors (Bassøe, 1956) has previously described insulin resistance, as determined by the insulin tolerance test (ITT) in 2 patients with Turner's syndrome and increased gonadotrophin excretion. One of these patients had a brother with Klinefelter's syndrome who also showed a similar response to ITT. In this patient, however, there was no increase in the excretion of gonadotrophins. Burt et al. (1954) reported that Klinefelter's syndrome and hyperplasia of the adrenals were frequently seen together. Lundstrom (1956) in his study of 5 patients with Turner's syndrome found one case with insulin resistance.

In rats ovariectomy is followed by a 500 % increase in the gonadotrophin concentration of the pituitary (Paesi et al., 1955). Furthermore, it has been shown that following castration, at least in the rat, there is an increase in the basophile elements of the pituitary gland (Gushing, 1932). It is possible that the gonadal dysgenesis in young

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J. Starup, J. Philip and V. Sele


This report describes in detail the histological and hormonal findings in a patient with Turner's syndrome (45,XO) and a patient with premature menopause (46,XX), who both conceived after withdrawal or reduction of substitution therapy with oestrogens. The aetiology of severe hypergonadotrophic ovarian failure is discussed, and theories regarding a possible relationship between the oestrogen treatment and subsequent pregnancy are hypothesized.

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Jean-Claude Reiter, Margareta Craen and Guy Van Vliet


A decreased growth hormone response to various secretagogues has been described in Turner's syndrome, but the mechanisms responsible for this decrease are unknown. Seventeen prepubertal girls with Turner's syndrome (age 6.4 to 15.7 years; height −0.2 to −5.4 sd, bone age −3.7 to −0.3 sd; weight 93 to 169% of ideal body weight) underwent a stimulation test with GHRH (0.5 μg/kg). Plasma GH and prolactin were measured by radioimmunoassay from −30 to +120 min and insulin-like growth factor-I at time 0. These values were compared with those observed in lean children with constitutional short stature. Peak plasma GH after GHRH was 17.0±3.6 μg/l (mean±sem), significantly lower (p<0.001) than in the short lean children (39.2±5.1 μg/l. In Turner's syndrome patients, the peak GH value was negatively correlated with the percentage of ideal body weight (r=−0.58, p<0.02) and of body fat (r=−0.59, p<0.02). Plasma prolactin levels in Turner's syndrome did not rise after GHRH and showed a normal circadian variation, from 8.0±1.0 μg/l at 08.30 h to 5.0±0.7 μg/l at 11.00 h (mean ±sem). Mean (±sem) baseline plasma insulin-like growth factor-I concentrations was 0.88±0.14 kU/l, higher than in the short lean children (0.49±0.08 kU/l, p<0.05). We conclude that the decreased GH response to GHRH of girls with Turner's syndrome is related, at least in part, to their excess body weight and fat and is associated with higher IGF-I levels than in short lean children.

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Andreas Mueller and Louis Gooren


To assess the risk of development of hormone-related tumors in transsexuals receiving treatment with cross-sex hormones.


Description of cases of transsexuals who have developed a hormone-related malignancy observed in their own clinic or reported in the literature. Recommendations for early diagnosis and prevention are presented.


Review of the literature in PubMed.


In male-to-female transsexuals receiving estrogen administration, lactotroph adenomas, breast cancer, and prostate cancer have been reported. In female-to-male transsexuals receiving treatment with testosterone, a single case of breast carcinoma and several cases of ovarian cancer have been reported. So far endometrial cancer has not been encountered though it remains a potential malignant development.


There are so far only a few cases of hormone-related cancer in transsexuals. There may be an underreporting. The probability of a hormone-related tumor increases with the duration of exposure to cross-sex hormones and the aging of the population of transsexuals.

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E. Schober, H Frisch, F. Waldhauser and Ch. Bieglmayr

Abstract. The modulating effect of estrogen on GH secretion was studied in 22 patients with Turner's syndrome. Estrogen administration (0.5 μg/kg ethinylestradiol) for a period of 4 weeks resulted in a significant increase in basal GH concentrations (2.6 vs 4.8 μg/l, P< 0.01). The L-Dopa-stimulated GH concentrations were also significantly increased (P< 0.01), whereas no effect of estrogen substitution on GH responses to GHRH (1–44) and Sm-C levels was seen. Our findings demonstrate a priming effect of estrogen on GH secretion in patients with Turner's syndrome. These patients generally lack the puberty-associated rise in GH secretion, which might be due to ovarian failure and the concomitant estrogen deficiency.

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CH Gravholt

Turner syndrome is one of the more common genetic disorders, associated with abnormalities of the X chromosome, and occurring in about 50 per 100 000 liveborn girls. Turner syndrome is usually associated with reduced adult height, gonadal dysgenesis and thus insufficient circulating levels of female sex steroids, and infertility. A number of other signs and symptoms are seen more frequently with the syndrome. Morbidity and mortality are increased. The average intellectual performance is within the normal range. A number of recent studies have provided new insights with respect to epidemiology, cardiology, endocrinology and metabolism. Treatment with GH during childhood and adolescence allows a considerable gain in adult height, although very-long-term consequences of this treatment are not clear. Puberty has to be induced in most cases, and female sex hormone replacement therapy is given during the adult years. The proper dose of hormone replacement therapy (HRT) has not been established, and, likewise, benefits and/or drawbacks from HRT have not been thoroughly evaluated. Since the risk of cardiovascular and endocrinological disease is clearly elevated, proper care during adulthood is emphasized. In summary, Turner syndrome is a condition associated with a number of diseases and conditions which are reviewed in the present paper.

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J. Lindsten, E. Cerasi, R. Luft and G. Hultquist


The plasma insulin response to prolonged glucose infusion was studied in 14 patients with Turner's syndrome having a normal intravenous glucose tolerance test. The insulin response was in most instances delayed and diminished in spite of a higher than normal blood glucose level.

The fasting plasma growth hormone (HGH) level was elevated, and hyperglycaemia induced a paradoxical increase in plasma HGH in most of the patients. The HGH response to hypoglycaemia was normal.

Oestrogen replacement accentuated the paradoxical HGH reaction to hyperglycaemia while the insulin response diminished further.

In contrast to earlier studies no increase in the frequency of diabetes was found among the relatives of the Turner patients.

In one subject there was a definite hyperplasia of the islet tissue of the pancreas with signs of decreased activity of the β-cells, in a second one the amount of islet tissue was fairly high.

Possible relationships between the above findings are discussed.

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F. K. Beller, K. H. Krumholz and K. Zeininger

Schon seit langem ist bekannt, dass Oxytocin neben seiner wehenanregenden Wirkung auch einen Einfluss auf die Lactation ausübt. Ott & Scott konnten 1910 zeigen, dass die intravenöse Injektion von Hypophysenhinterlappenextrakt wenige Sekunden nach der Injektion einen Milchfluss auslöst. Diese Versuche wurden später mehrfach bei verschiedenen Tierarten wiederholt und bestätigt (Schäfer & MacKenzie, 1911, MacKenzie, 1911, Turner & Slaughter, 1930, Whittlestone, 1952, Petersen, 1944, Folley, 1947, Newton & Newton, 1948). Turner & Cooper (1941) gaben an, dass Oxytocinpräparate mit hohem Vasopressingehalt wirksamer seien als reine Oxytocinpräparate, Vasopressin sogar letzteren überlegen sein soll. Im Gegensatz dazu konnten Van Dyke et al. (1955) und Konzett et al. (1956) zeigen, dass das synthetisch von du Vigneaud (1953) hergestellte Oxytocin, das weniger als 1 % Vasopressin-Aktivität enthält, ebenso wirksam war, wie Gesamtextrakte mit hohem Vasopressingehalt (Cross & Van Dyke, 1953). Während nach Van Dyke 100 Pharmakopoe-Einheiten Oxytocin auch im »milk-ejection«-Test 100 Internationalen Einheiten entsprechen, entfalten 100