ENDOCRINE STUDIES AND SUCCESSFUL TREATMENT IN A PATIENT WITH TRUE HERMAPHRODITISM

1979 ◽  
Vol 91 (1) ◽  
pp. 184-192
Author(s):  
Evangelina Valdés ◽  
Carlos Fernández del Castillo ◽  
Raul Gutiérrez ◽  
Fernando Larrea ◽  
Martha Medina ◽  
...  
Keyword(s):  
Chromosome Complement ◽  
Reproductive Function ◽  
External Genitalia ◽  
Serum Testosterone ◽  
Serum Levels ◽  
Normal Children ◽  
Serum Lh ◽  
Genital Ambiguity ◽  
And Gender ◽  
Lh Rh

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.

Endocrine and immunogenetic evaluation of an XX male infant with perineoscrotal hypospadias

1985 ◽  
Vol 108 (3) ◽  
pp. 421-427 ◽  
Author(s):  
S. Kofman-Alfaro ◽  
E. Valdés ◽  
J. Terá ◽  
S. S. Wachtel ◽  
B. Chávez ◽  
...  
Keyword(s):  
Specific Binding ◽  
Chromosome Complement ◽  
External Genitalia ◽  
Male Infant ◽  
Serum Testosterone ◽  
Normal Activity ◽  
Significant Rise ◽  
Cultured Fibroblasts ◽  
Genital Ambiguity ◽  
Xx Male

Abstract. To clarify the origin of the genital ambiguity occasionally associated with the XX male syndrome, a series of endocrinological studies were undertaken in an affected 6 months old infant with perineoscrotal hypospadias. The patient fulfilled all the diagnostic criteria of the syndrome: the testes were descended bilaterally, the Mullerian derivatives were absent, the 46,XX chromosome complement was ascertained in different cell lines, and male levels of H-Y antigen were detected in cultured skin fibroblasts. Circulating gonadotrophin levels and pituitary LRH responsiveness were within normal limits for the age group. Serum testosterone (T) levels were normal, and gonadal stimulation with hCG caused a significant rise on serum T. Incubations of [3H]T with fibroblasts from genital skin revealed normal activity of steroid 5α-reductase. Moreover, normal concentrations of thermostable cytosol androgen receptors were revealed in cultured fibroblasts. Altogether the results indicated that ambiguity of the external genitalia in this patient was the result of neither abnormal T biosynthesis, peripheral A-ring T reduction, nor androgen intracellular specific binding, and suggested that the nature of the imcomplete virilization could be a non-endocrine independent event associated to this disorder. The data are also consistent with the notion that testicular impairment observed in adult XX males develops later in life.

Serum LH and FSH Responses to Synthetic LH-RH in Normal Infants, Children and Patients With Turner's Syndrome

PEDIATRICS ◽  
1974 ◽  
Vol 54 (4) ◽  
pp. 470-475
Author(s):  
Selzo Suwa ◽  
Hatae Maesaka ◽  
Ichiro Matsui
Keyword(s):  
Normal Subjects ◽  
Serum Levels ◽  
Young Female ◽  
Normal Female ◽  
Turner's Syndrome ◽  
Turner’S Syndrome ◽  
Normal Children ◽  
Serum Lh ◽  
Female Children ◽  
Lh Rh

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.

EFFECTS OF MATING AND INJECTION OF LUTEINIZING HORMONE RELEASING HORMONE ON SERUM LUTEINIZING HORMONE AND TESTOSTERONE CONCENTRATIONS IN RABBITS

1978 ◽  
Vol 76 (3) ◽  
pp. 417-425 ◽  
Author(s):  
C. A. BLAKE ◽  
PATRICIA K. BLAKE ◽  
NANCY K. THORNEYCROFT ◽  
I. H. THORNEYCROFT
Keyword(s):  
Luteinizing Hormone ◽  
Serum Testosterone ◽  
Serum Levels ◽  
Transient Increase ◽  
Marked Rise ◽  
Luteinizing Hormone Releasing Hormone ◽  
Releasing Hormone ◽  
Serum Lh ◽  
Before And After ◽  
Lh Rh

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.

PITUITARY-GONADAL FUNCTION IN MALE PATIENTS WITH MYOTONIC DYSTROPHY - SERUM LUTEINIZING HORMONE, FOLLICLE STIMULATING HORMONE AND TESTOSTERONE LEVELS AND HISTOLOGICAL DAMAGE OF THE TESTIS

1977 ◽  
Vol 84 (2) ◽  
pp. 382-389 ◽  
Author(s):  
Ryoyu Takeda ◽  
Misao Ueda
Keyword(s):  
Myotonic Dystrophy ◽  
Serum Testosterone ◽  
Serum Levels ◽  
Seminiferous Tubules ◽  
Reciprocal Relation ◽  
Logarithmic Scale ◽  
Serum Luteinizing Hormone ◽  
Serum Lh ◽  
Male Patients ◽  
Lh Rh

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.

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 ◽  
...  
Keyword(s):  
Serum Testosterone ◽  
Serum Levels ◽  
High Serum ◽  
Normal Serum ◽  
Constant Infusion ◽  
Normal Female ◽  
Adrenocortical Adenoma ◽  
Serum Lh ◽  
The Right ◽  
Lh Rh

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.

ABNORMAL RESPONSE OF LUTEINIZING HORMONE, FOLLICLE STIMULATING HORMONE AND TESTOSTERONE TO LUTEINIZING HORMONE-RELEASING HORMONE IN CHRONIC RENAL FAILURE

1978 ◽  
Vol 87 (3) ◽  
pp. 467-475 ◽  
Author(s):  
Koichi Hasegawa ◽  
Yoshiki Matsushita ◽  
Kenzo Hirai ◽  
Seima Otomo ◽  
Teruo Okamoto ◽  
...  
Keyword(s):  
Renal Failure ◽  
Chronic Renal Failure ◽  
Luteinizing Hormone ◽  
Healthy Subjects ◽  
Serum Testosterone ◽  
Serum Levels ◽  
Normal Range ◽  
Serum Lh ◽  
Lh Rh ◽  
Male Subjects

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.

ANNUAL RHYTHMS OF LUTEINIZING HORMONE, FOLLICLE-STIMULATING HORMONE, PROLACTIN AND TESTOSTERONE IN THE SERUM OF MALE RHESUS MONKEYS

1979 ◽  
Vol 83 (2) ◽  
pp. 131-139 ◽  
Author(s):  
W. BECK ◽  
W. WUTTKE
Keyword(s):  
Rhesus Monkeys ◽  
Serum Testosterone ◽  
Serum Levels ◽  
Serum Prolactin ◽  
First Year ◽  
Thyrotrophin Releasing Hormone ◽  
Testosterone Levels ◽  
Serum Lh ◽  
June July ◽  
Male Rhesus

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.

scholarly journals Reproductive function in men affected by X-linked adrenoleukodystrophy/adrenomyeloneuropathy

2012 ◽  
Vol 166 (2) ◽  
pp. 291-294 ◽  
Author(s):  
T J Stradomska ◽  
J Kubalska ◽  
R Janas ◽  
A Tylki-Szymańska
Keyword(s):  
Elevated Serum ◽  
Reproductive Function ◽  
Serum Testosterone ◽  
Chain Fatty Acid ◽  
Resonance Imaging ◽  
Normal Range ◽  
Long Chain Fatty Acid ◽  
Chromatography Method ◽  
Adult Men ◽  
Serum Lh

BackgroundX-linked adrenoleukodystrophy (X-ALD) is the most frequent, severely neurodegenerative, clinically heterogeneous peroxisomal disorder, the signs of which are a consequence of myelin, adrenal cortex, and testes impairment.ObjectiveWe studied testosterone, LH, and FSH levels in X-ALD/adrenomyeloneuropathy (AMN) patients. We evaluate the ability to procreate of these patients by analysis of pedigree and family screening by detection of very long-chain fatty acid (VLCFA) levels.Subject and methodsSeventeen patients with X-ALD/AMN (16 with AMN and one asymptomatic) aged 24–48 (mean±s.d., 34.7±5.9) years, were identified based on the clinical picture, magnetic resonance imaging, and the presence of increased serum VLCFA levels. Nine X-ALD/AMN patients' daughters, mean ages ±s.d.=7.7±3.8 years, were identified as heterozygote by elevated VLCFA levels.Serum VLCFA levels were determined as ester derivatives by a gas chromatography method. Serum testosterone, LH, and FSH levels in X-ALD/AMN patients were detected by IRMAs.ResultsSerum testosterone levels were at the lowest levels of normal range but serum LH and FSH concentrations were increased in 57.1 and in 42.9% of X-ALD/AMN patients respectively. Among the 11 investigated of X-ALD/AMN married adult men, nine had produced offspring, a total of 13 children. All patients' daughters showed elevated serum VLCFA at heterozygote levels.ConclusionIn this study, we report that in a group of X-ALD/AMN married adult men, we did not find a significant decrease in fertility compared with the Polish population (18.2 vs 15%).

The contribution of corticosterone and testosterone to the suppression of serum LH in hyperprolactinaemic adult male rats with pituitary transplants

1987 ◽  
Vol 113 (1) ◽  
pp. 111-116 ◽  
Author(s):  
R. F. A. Weber ◽  
M. P. Ooms ◽  
J. T. M. Vreeburg
Keyword(s):  
Adult Male ◽  
Serum Testosterone ◽  
Serum Levels ◽  
Male Rats ◽  
Male Rat ◽  
Serum Concentrations ◽  
Testosterone Secretion ◽  
Accessory Sex Glands ◽  
Serum Lh ◽  
Stimulation Of

ABSTRACT The effects of hyperprolactinaemia on serum levels of LH were investigated in adult male rats of the R × U strain. Hyperprolactinaemia was induced by three pituitary grafts under the kidney capsule, transplanted on day 0 of each experiment. Special attention was paid to the contribution of prolactin-stimulated testes, adrenals and corticosterone. In experiment 1, hyperprolactinaemia significantly reduced the serum concentrations of LH in intact rats. In spite of a significant increase in the serum levels of corticosterone, serum testosterone was not significantly affected by hyperprolactinaemia. The weights of both the adrenals and accessory sex glands were significantly increased at autopsy. In experiment 2, treatment with 10 mg corticosterone s.c. daily from day 14 to day 28 after pituitary grafting significantly reduced serum levels of both LH and testosterone. The suppression of testosterone in the hyperprolactinaemic corticosterone-treated animals was significantly less than in the corticosterone-treated control animals. The weights of the accessory sex glands were significantly increased in the hyperprolactinaemic animals. In experiment 3, rats were adrenalectomized and half of them were substituted with corticosterone. Serum testosterone levels significantly increased in both hyperprolactinaemic adrenalectomized rats and in adrenalectomized corticosterone-treated animals without any significant effect on serum LH. Again the weights of the accessory sex glands were significantly increased in the hyperprolactinaemic animals. In experiment 4, rats were adrenalectomized, gonadectomized and corticosterone treated on day 0 and then implanted with a 2, 1·5 or 1 cm silicone elastomer capsule containing testosterone. On day 28 after pituitary grafting, LH levels were significantly suppressed in animals with a 2 or 1·5 cm testosterone implant. The weights of the accessory sex glands were not increased in the hyperprolactinaemic animals. These results show that in the male rat the inhibitory effects of hyperprolactinaemia on serum LH levels may be due to (1) increased sensitivity of the hypothalamic-pituitary axis to the negative feedback action of testosterone by prolactin and by the prolactin-stimulated corticosterone secretion and (2) stimulation of testicular testosterone secretion by prolactin, which can also explain the increased weights of the accessory sex glands. Even in the presence of high serum concentrations of corticosterone, stimulation of testicular testosterone secretion by prolactin was observed. J. Endocr. (1987) 113,111–116

ABSENCE OF POSITIVE FEEDBACK EFFECT OF OESTROGEN ON LH RELEASE IN PATIENTS WITH TESTICULAR FEMINIZATION SYNDROME

1978 ◽  
Vol 87 (2) ◽  
pp. 259-267 ◽  
Author(s):  
Toshihiro Aono ◽  
Akira Miyake ◽  
Takayuki Kinugasa ◽  
Keiichi Kurachi ◽  
Keishi Matsumoto
Keyword(s):  
Sex Differentiation ◽  
Serum Testosterone ◽  
Normal Male ◽  
Testicular Feminization ◽  
Serum Lh ◽  
Exogenous Oestrogen ◽  
Lh Release ◽  
Normal Males ◽  
Lh Rh

ABSTRACT The response of serum LH to exogenous oestrogen administration was studied in 5 patients with testicular feminization syndrome (TFS). The serum LH levels were elevated in all the patients, while serum testosterone levels were within the normal male range. Serum FSH levels were elevated in 4 patients and normal in one patient. Intravenous administration of 100 μg of LH-RH provoked a further increase in both LH and FSH. Following intravenous injection of 20 mg of conjugated oestrogen (Premarin®), the LH levels were serially determined until 120 h in TFS patients, 5 normal males, and 10 normal females during the mid-follocular phase (D7-9). Both TFS patients and normal males showed no LH release following oestrogen injection in contrast to normal females who displayed a significant increase in LH with a peak at 48 to 56 h after the injection. These results seem to suggest that the insensitivity of the hypothalamus to androgen in TFS patients do not affect the sex differentiation of the hypothalamus. The possible role of oestradiol conversion from testosterone in the hypothalamus is discussed.

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