A GONADOTROPHIN-RESPONSIVE VIRILIZING ADRENAL TUMOUR IDENTIFIED AS A MIXED GANGLIONEUROMA AND ADRENOCORTICAL ADENOMA

1978 ◽  
Vol 89 (4) ◽  
pp. 701-709 ◽  
Author(s):  
Hisatoshi Takahashi ◽  
Kenichi Yoshizaki ◽  
Hideyuki Kato ◽  
Toshihisa Masuda ◽  
Goro Matsuka ◽  
...  

ABSTRACT A 38-year-old gravida 2, para 1 woman, who had suffered from virilization and amenorrhoea for 8 years was examined. She had peripheral serum testosterone (T) levels of 3.9–8.7 ng/ml (normal level: 0.32 ± 0.09 ng/ml) with normal serum levels of dehydroepiandrosterone (DHA), aldosterone and cortisol (F) and normal urinary 17-ketosteroids (17-KS). Dexamethasone (DXM 2 mg/day for 5 days) suppressed the serum F level adequately, but reduced the high T levels only slightly. The administration of commercial hCG 6000 IU for 3 days increased both the serum T levels (5.68 to 9.83 ng/ml) and the serum DHA levels (4.9 to 9.8 ng/ml, normal range 4–6 ng/ml), but synthetic ACTH-Z (Cortrosyn-Z®, 1 mg, Organon) did not affect the serum T level. The basal serum LH level was abnormally low, and constant infusion of synthetic LH-RH (200 μg/5 h) resulted in good responses of serum LH and FSH and increase in the serum T level from 5.2 to 9.1 ng/ml. Computerized tomography, ultrasonography and pneumo-retroperitoneumroentgenography showed the presence of a large tumour in the right adrenal. The tumour was a circumscribed irregularly lobulate mass, measuring 16×8×8 cm and weighing 500 g. Histologically, it was identified as a ganglioneuroma containing numerous scattered groups of large polyhydral cells similar to adrenocortical cells, forming islets in some places. After removal of the tumour from the right adrenal, the high serum T level decreased to the normal female level in 24 h.

1979 ◽  
Vol 91 (1) ◽  
pp. 184-192
Author(s):  
Evangelina Valdés ◽  
Carlos Fernández del Castillo ◽  
Raul Gutiérrez ◽  
Fernando Larrea ◽  
Martha Medina ◽  
...  

ABSTRACT A 12-year old, 46 XX true hermaphrodite born with genital ambiguity was studied and successfully treated. The serum LH and FSH profile resembled that of a pubertal normal individual, and LH-RH administration induced a normal LH response. Baseline testosterone serum levels were within the range for normal children. Exogenous HCG stimulation induced a significant serum testosterone increase up to values similar to those observed in normal post-pubertal males. Surgical examination disclosed the presence of bilateral ovotestis, normal Mullerian derivatives, epididymis, and vas deferens. A complete ovotestis with testicular predominance and the testicular portion of the contralateral ovotestis as well as the Wolffian derivatives, were removed. A further HCG stimulation 3 months after surgery, failed to induce serum testosterone increase. Spontaneous menarche was observed 6 months after surgery and ovulation was well documented. At present the patient has several characteristics of female sex including those of chromosome complement, gonad, internal and external genitalia, hormone levels and gender identity, thus demonstrating that treatment was successful and that reproductive function could be obtained. The finding of spontaneous ovulation following removal of the testicular portion suggests normal cyclic gonadotrophic release implying a difference between animal models and man in regard to hypothalamic virilization.


PEDIATRICS ◽  
1974 ◽  
Vol 54 (4) ◽  
pp. 470-475
Author(s):  
Selzo Suwa ◽  
Hatae Maesaka ◽  
Ichiro Matsui

Effects of LH-RH on LH and FSH release were studied in 26 normal children and six patients with Turner's syndrome (two of them showed 45 x karyotype and the others were mosaics). Synthetic LH-RH (2µg/kg of body weight) was given intramuscularly after an overnight fast. The increase of serum FSH level was significantly greater in normal female infants than male infants. A similar tendency was observed in normal female children aged 2 to 9 years. No sex difference was observed in the LH response to LH-RH in all the normal subjects Studied. The response of FSH release to LH-RH was significantly greater than that of LH in female infants and young female children. The responsiveness of LH to LH-RH gradually increased with advancing age. Basal serum levels of FSH rather than LH were high in the patients with Turner's syndrome. Again a much greater increase of serum FSH than of LH was noted after the administration of LH-RH to patients with Turner's syndrome.


1978 ◽  
Vol 76 (3) ◽  
pp. 417-425 ◽  
Author(s):  
C. A. BLAKE ◽  
PATRICIA K. BLAKE ◽  
NANCY K. THORNEYCROFT ◽  
I. H. THORNEYCROFT

The effects of coitus and injection of luteinizing hormone releasing hormone (LH-RH) on serum concentrations of LH, testosterone and dihydrotestosterone (17β-hydroxy-5α-androstan-3-one; DHT) were tested in male rabbits. Before experimentation, male and female rabbits were housed in individual cages in the same room. Male rabbits were then bled by cardiac puncture before and after placement with female rabbits or intravenous injection of LH-RH. Serum LH, testosterone and DHT were measured by radioimmunoassay. Sexual excitement (sniffing, chasing and mounting), with or without intromission, caused a marked rise in serum testosterone and DHT concentrations in only some of the bucks. These increases were accompanied or preceded by a small, transient increase in serum LH. In the rest of the bucks, sexual excitement with or without intromission had either no effect on serum levels of all three hormones, or only serum testosterone and DHT decreased during the collection period. Similar responses were measured in bucks which were housed in a room without does for 2–4 weeks before experimentation. Injection of 10, 30 or 100 ng or 50 μg LH-RH caused serum LH, testosterone and DHT to rise in all bucks tested, but the magnitude of the rises in serum testosterone and DHT were not related to the magnitude of the LH rise. In both mated and LH-RH-injected bucks, the rises in serum testosterone and DHT were greatest in animals with low initial testosterone and DHT values. Under the conditions of this study, the data suggest that: (1) serum testosterone and DHT rise in only some male rabbits after sexual excitement (with or without intromission), (2) the rises in serum testosterone and DHT are dependent on a small transient increase in serum LH and (3) sexual excitement is less likely to cause release of LH-RH in bucks with raised serum testosterone and DHT concentrations.


1977 ◽  
Vol 84 (2) ◽  
pp. 382-389 ◽  
Author(s):  
Ryoyu Takeda ◽  
Misao Ueda

ABSTRACT Variations in serum levels of LH and FSH after administration of synthetic LH-RH, and basal levels of serum testosterone were studied in 9 male patients with myotonic dystrophy. The degree of testicular damage, as determined histologically on biopsy specimens, was also studied. Results were as follows: 1) it was observed that both the basal and stimulated (maximal) levels of serum LH and FSH were significantly higher than levels found in 9 sex- and age-matched normal controls. 2) The basal level of serum testosterone was consistently lower in the patient group. One patient, however, showed a low normal level, which represented a statistically significant reciprocal relation to both LH and FSH levels as expressed on a logarithmic scale. 3) There was a significant trend which indicated that the higher the serum LH, FSH levels, or the lower the basal levels of serum testosterone, the more extensive was the damage to the seminiferous tubules. From these findings it was concluded that hypogonadism in patients with myotonic dystrophy, is characterized by the development of lesions in the seminiferous tubules.


1984 ◽  
Vol 105 (3) ◽  
pp. 308-313 ◽  
Author(s):  
David R. Mann ◽  
Michael S. Blank ◽  
R. Sridaran ◽  
V. Daniel Castracane ◽  
Charles Eldridge ◽  
...  

Abstract. The objective of this study was to determine whether anti-oestrogens (nafoxidine, MER-25) would block the suppressive effects of ACTH on gonadotrophin secretion in immature rats. Female rats were castrated at 25–26 days of age, and an Alzet osmotic minipump containing ACTH (1–24) or saline was implanted in each animal. ACTH was administered at a rate of 1 IU/day by constant infusion. Beginning on the day of surgery, animals were injected daily for 5 days with 0.25, 5 or 25 μg/100 g body weight of nafoxidine or 5 mg MER-25 and sacrificed on the sixth day following castration. ACTH lowered serum LH concentrations and increased pituitary LH levels. Serum androstenedione concentrations were more than two times greater in ACTH-infused than in control rats, but serum oestrone levels were not affected. Serum testosterone and oestradiol concentrations in ACTH-infused rats remained below levels of detection. Administration of 0.25 μg of nafoxidine prevented the suppressive effects of ACTH on serum LH. Serum levels of LH in these animals were comparable to saline-treated controls (418 ± 94 vs 443 ± 73 ng/ml). The two higher doses of nafoxidine and MER-25 were ineffective in suppressing the actions of ACTH on serum LH. MER-25 reduced serum LH values in both controls and ACTH-infused rats. Serum FSH concentrations were not altered by ACTH or nafoxidine treatment. MER-25 elevated pituitary FSH concentrations in both control and ACTH-infused rats. These data suggest that the inhibitory effect of ACTH on LH secretion in immature rats is mediated by an oestrogenic steroid, since this action can be blocked by simultaneous treatment with a low dose of the anti-oestrogen, nafoxidine.


1978 ◽  
Vol 87 (3) ◽  
pp. 467-475 ◽  
Author(s):  
Koichi Hasegawa ◽  
Yoshiki Matsushita ◽  
Kenzo Hirai ◽  
Seima Otomo ◽  
Teruo Okamoto ◽  
...  

ABSTRACT Serum levels of LH, FSH, testosterone and oestradiol were measured by radioimmunoassay in 10 healthy subjects and 7 undialysed and 15 dialysed patients with chronic renal failure. The basal level of serum LH was significantly higher in patients with chronic renal failure than in healthy subjects. The basal level of serum FSH in male subjects was significantly higher in dialysed patients with chronic renal failure than in healthy subjects. Regarding testosterone secretion in male subjects and oestradiol secretion in female subjects, it was shown that the basal level of serum testosterone was significantly lower in patients with chronic renal failure than in healthy subjects, and that serum oestradiol levels were within the normal range in most of the patients with chronic renal failure. Serum levels of LH, FSH and testosterone were determined after iv injection of 100 μg of LH-RH in three groups of subjects. Responses of serum LH and FSH to LH-RH were exaggerated and prolonged in patients with chronic renal failure compared with healthy subjects. The peaks of serum LH and FSH were observed 30 min after LH-RH injection in healthy subjects while those in chronic renal failure appeared at 60 to 120 min. The levels of serum testosterone after LH-RH injection did not change within 120 min. These findings may indicate that the pituitary gonadotrophin response to LH-RH is abnormal and that the turnover of LH, FSH and LH-RH is decreased in patients with chronic renal failure.


PEDIATRICS ◽  
1983 ◽  
Vol 72 (3) ◽  
pp. 384-389
Author(s):  
Harold K. Marder ◽  
Laxmi S. Srivastava ◽  
Stephen Burstein

Serum gonadotropin and testosterone concentrations were measured in ten peripubertal boys to assess the effects of uremia on pubertal maturation. Serum luteinizing hormone (LH) concentrations were elevated for stage of puberty in eight boys, whereas in most boys serum follicle-stimulating hormone and testosterone concentrations were normal. Serum LH concentrations correlated with the severity of uremia. LH levels declined when measured 1 year after the initial measurements in four boys who received renal allografts, but were further elevated in two boys who were treated conservatively. Elevated serum LH concentrations in the presence of normal serum testosterone concentrations imply limited testicular sensitivity to the effects of LH in these peripubertal boys, as has been documented for adult men with chronic renal failure. Alternatively, there may be accumulation of an immunoreactive LH molecule that lacks bioactivity. A testicular dysfunction may explain the pubertal delay experienced by some uremic adolescent boys.


1979 ◽  
Vol 83 (2) ◽  
pp. 131-139 ◽  
Author(s):  
W. BECK ◽  
W. WUTTKE

Six male rhesus monkeys were kept under rigidly controlled conditions for 1–2 years. During August of the first year a thyrotrophin releasing hormone (TRH) test was performed on each of the monkeys by giving 10 μg TRH as a bolus injection. Significantly increased serum prolactin levels occurred 15 min after the injection. After a training period of 2 months, during which blood samples were collected every other day by puncture of the saphenous vein, blood was collected three times a week for 14 months. Serum levels of prolactin, LH, FSH and testosterone were measured by radioimmunoassay. Mean serum prolactin levels increased significantly during June, July and August in all six animals. Peak levels were observed in August and September and then levels declined gradually to reach a minimum in April and May. Mean serum testosterone levels closely paralleled the annual pattern of prolactin. Mean serum LH levels significantly decreased during the time when mean serum prolactin and testosterone levels were increasing and they increased again at the time of decreasing mean prolactin levels, i.e. mean serum LH and prolactin were negatively correlated. In individual monkeys, however, a rigid negative correlation between serum prolactin and LH could not be demonstrated. Mean serum FSH levels did not change significantly.


1979 ◽  
Vol 90 (2) ◽  
pp. 328-335 ◽  
Author(s):  
B. Baranowska ◽  
S. Zgliczyński

ABSTRACT In order to elucidate the mechanism of disturbances of gonadal hormones secretion in anorexia nervosa 14 female patients were investigated. A control group also consisted of 14 women of the same age. The serum LH, progesterone, oestrogens: oestrone + oestradiol (Oe1 + Oe2), oestriol (Oe3) and testosterone were determined by radioimmunological methods. In patients with anorexia nervosa the serum testosterone and Oe3 concentrations were dramatically elevated, whereas LH, progesterone and Oe1 + Oe2 were decreased as compared with the control group. Considerable weight gain induced by cyproheptadine treatment caused a normalization of the serum testosterone and Oe3 concentrations in all the patients. A negative correlation between the testosterone level and the deficit in body weight was observed. The mechanism causing the dramatically high serum testosterone concentration in the female patients with anorexia nervosa is discussed.


2005 ◽  
Vol 8 (1) ◽  
pp. 92-97 ◽  
Author(s):  
Sevgi Bakaris ◽  
Sefa Resim ◽  
Nurdan Tunali

We report the case of a 17-year-old male with a testicular tumor and high serum levels of α-fetoprotein. The patient was treated with surgery followed by combination chemotherapy with bleomycin, etoposide, and cisplatin. Histologic examination showed features of a mixed germ cell tumor composed of mature teratoma, immature teratoma, embryonal carcinoma, yolk sac tumor, and polyembryoma. He is currently well, and his serum levels of α-fetoprotein have been normal more than 5 months after treatment. His brother, aged 17 years at the time, had a similar tumor removed from the right testicle 5 years previously.


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