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At least 19 recordsLinked to original sources

Fertile eunuch syndrome versus classic hypogonadotrophic hypogonadism.

The functioning of the hypothalamo-pituitary-target organs axis was assessed in 3 patients with 'fertile eunuch' syndrome (FE) and 6 patients with 'classic' hypogonadotrophic hypogonadism (HH) with or without hyposmia. Both groups of patients did not differ from each other with regard to basal serum prolactin levels, pituitary growth hormone and thyrotrophin reserve and the thyroid or adrenal gland function. Both groups differed, however, with respect to the hypothalamo-pituitary-gonadal function: 1. the pituitary LH response to exogenous LH-RH was (low)-normal in FE and blunted in HH; 2. the basal FSH levels were normal in FE and undetectable in HH; 3. the basal LH levels were normal in FE and 3/6 patients with HH and low in the remaining three; 4. the basal and HCG stimulated plasma testosterone concentrations were significantly higher in FE than HH. The data suggest that FE represents a less severe form of LH-RH deficiency, rather than a distinct disorder.

Adult

The fertile eunuch syndrome. An isolated leydig-cell failure?

In two patients exhibiting eunuchoid features in association with normal sized testes and complete spermatogenesis concommittant with only occasional Leydig cells between the tubuli, (proven by testicular biopsy) an attempt was made to elucidate the factors leading to this condition. Both patients responded with significant rise in both plasma FSH and LH after administration of synthetic GnRH indicating pituitary responsiveness. However, no rise in either FSH or LH could be observed after administration of clomiphene citrate during three weeks of treatment indicating hypothalamic unresponsiveness to chemical stimuli. Although plasma testosterone levels rose significantly after administration of Human Chorionic Gonadotropin, estradiol remained unchanged during three weeks of HCG administration. A hypothesis is discussed which defines this syndrome in these two cases as primary Leydig cells failure, expressed in the inability of these to transform testosterone into estrogens, thus depriving pituitary and hypothalamus from a proper steroidal milieu necessary to adequate functioning.

Adult

Moebius syndrome in Kallmann syndrome.

A girl born with congenital paresis of cranial nerves III, IV, and VII (Moeblus syndrome) subsequently developed a progressive peripheral neuropathy. There was suggestive evidence of a familial neuropathy with autosomal dominant inheritance in three family members. The patient also had hypogonadotrophic hypogonadism and anosmia (Kallmann syndrome).

Abducens Nerve

A diploid-triploid human mosaic with cytogenetic evidence of double fertilization.

The karyotype 46,XX/69,XXY was found in a 13-year-old mentally subnormal patient with club feet, strabismus, eunuchoid habitus, small penis, midscrotal urethrovaginal opening, small descended left testis, and small undescended right testis; no ovarian tissue could be found at laparotomy. Triploid:diploid cell ratios were 60:40 and 4:96 in skin fibroblasts and curculating lymphocytes, respectively. In the triploid line, two of the no. 13 chromosomes had unusually large satellites and one of the no. 22 chromosomes had a brightly fluorescent zone on its short arms. The patient's father was heterozygous for both these autosomal markers; the mother carried neither marker. This, together with the single Y, indicated that the extra haploid set was derived from the father. Of several possible mechanisms, we favor the suggestion that double fertilization occurred; one sperm nucleus immediately fused with the egg nucleus producing the diploid line; the second sperm nucleus was incorporated later into one of the two cells resulting from the first division of the zygote, producing the triploid line.

Adolescent

Analysis of body height in 829 patients with different forms of testicular pathology.

Analysis of body height was performed in the control group of 500 well-fertile men and in 829 patients with various forms of testicular pathology such as hypoplasia of testicles, unilateral and bilateral cryptorchism, hypogonadotrophic eunuchodism, and Klinefelter's syndrome. A statistically greater average height was found in bilateral cryptorchids, unilateral cryptorchids with an adequate spermiogenesis, hypogonadotrophic eunuchoids and chromatin-positive men. The greatest difference, when compared with the control group, was found in patients with a Klinefelter's syndrome. The authors hold the view that the plasmatic testosterone values are not the only factor conditioning the termination of the process of growth into height in males. Importance must also be given to the lowered sensibility of epiphysial growth zones to male sex hormone; this reduced sensibility is especially to be seen in chromatin-positive men.

Adult

A case of hypogonadotropic eunuchoidism associated with familiar periodic paralysis--induction of paralytic attacks by testosterone administration.

A case report was made of a 24-year-old man suffering from hypogonadotropic eunuchoidism associated with a hypokalemic type of familiar periodic paralysis. The attacks were observed only during the period of continual administration of the oil solution of 115.7 mg of testosterone enanthate and 20.0 mg of testosterone propionate, intramuscularly as a depot. From the findings on this patient, it was concluded that testosterone might possibly play a role in the occurrence of attacks in the case of periodic paralysis.

Adult

Induction of spermatogenesis in hypogonadotrophic hypogonadism.

A young male who presented with isolated bihormonal gonadotrophin deficiency is described, Basal levels of LH and FSH were low and there was no response to clomiphene citrate or LHRH. The remaining anterior pituitary function was intact. The administration of a combination of human menopausal gonadotrophin and human chorionic gonadotrophin caused testicular maturation with spermatogenesis and full androgenization. The patient was able to father a child.

Adult

Oestrone, oestradiol and testosterone in normal and hypogonadal men following LH-RH or HCG stimulation.

oestrone, oestradiol-17beta and testosterone in serum were measured during a LH-RH infusion test and a Leydig cell function test with HCG in healthy men and in patients with primary or secondary hypogonadism. Following infusion of LH-RH increases of LH (+112%), oestradiol (+62%) and testosterone (+51%) were observed in normal men, while oestrone remained unchanged. Of the patients only those with Klinefelter's syndrome showed a significant increase in testosterone and oestradiol after LH-RH infusion. During the Leydig cell function test oestradiol and testosterone exhibited a largely parallel pattern under normal and pathological conditions as well as in subjects with experimental hypogonadism. Augmentations of plasma oestrone were usually smaller than those for oestradiol.

Chorionic Gonadotropin