A Controlled Study of the Psychopathology and Physical Measurements of Klinefelter's Syndrome

1969 ◽  
Vol 115 (521) ◽  
pp. 443-448 ◽  
Author(s):  
H. Hunter

Klinefelter, Reifenstein and Albright (13) in 1942 collected and described nine cases of a new clinical syndrome, later given the eponym Klinefelter's Syndrome, which appeared at puberty in males and was characterized by gynaecomastia, hypogonadism, aspermatogenesis, increased follicle-stimulating hormone (F.S.H.) and normal or low 17-ketosteroid urinary excretion.

1986 ◽  
Vol 42 (2) ◽  
pp. 153-157 ◽  
Author(s):  
Antonio Bellastella ◽  
Tuilio Criscuolo ◽  
Antonio A. Sinisi ◽  
Sergio Iorio ◽  
Antonietta M. Sinisi ◽  
...  

1994 ◽  
Vol 130 (1) ◽  
pp. 60-64 ◽  
Author(s):  
Jantine JG Hoorweg-Nijman ◽  
Hester M Havers ◽  
Henriette A Delemarre-van de Waal

Hoorweg-Nijman JJG, Havers HM, Delemarre-van de Waal HA. Effect of human chorionic gonadotrophin (hCG)/follicle-stimulating hormone treatment versus hCG treatment alone on testicular descent: a double-blind placebo-controlled study. Eur J Endocrinol 1994;1 30:60–4. ISSN 0804–4643 The medical treatment of retentio testis remains controversial because of ineffectiveness and/or adverse events. Follicle-stimulating hormone (FSH) seems to influence the spontaneous descent of the testis; furthermore, it induces luteinizing hormone (LH) receptors. Therefore, we performed a double-blind placebo-controlled study to investigate the effect of FSH with human chorionic gonadotrophin (hCG) versus hCG alone in retentio testis patients. Twenty-two boys with retentio testis were investigated, excluding retractile testis. Group A (N= 14: four with bilateral and 10 with unilateral retentio testis; mean age 3.1 5 years) was treated with 150 IU of FSH twice a week for 2 weeks followed by 1 50 IU of FSH and 250 IU of hCG (half the recommended World Health Organization dose) twice a week for another 4 weeks. Group B (N = 8: two with bilateral and six with unilateral retentio testis: mean age 3.3 years) was treated with 250 IU of hCG twice a week for 6 weeks. Testicular position, volume and consistency as well as the appearance of the scrotum and the penile length were determined at the start of the treatment as well as at weeks 2.4.6 and 12 by two independent investigators. Blood investigation consisted of measurements of LH, FSH, testosterone and sex hormone-binding globulin. Successful descent was considered when the testis reached a mid- or low scrotal position. In group A, 6/18 testes descended successfully. In group B, 6/10 testes descended. Of the unsuccessfully treated patients, six of group A and three of group B underwent surgery. Of these patients, 6/8 testes of group A and all testes of group B showed anatomical abnormalities, which could explain the lack of hormonal response. There were no significant differences in hormonal parameters between the two groups. In both groups no serious adverse events were mentioned or observed. In conclusion, half the recommended WHO dose of hCG is sufficient to reach successful descent in 43% of treated patients, with no serious adverse events; this response rate is in agreement with the literature. Follicle-stimulating hormone does not seem to have an additional effect on the success rate, and most of the unsuccessfully treated patients showed anatomical abnormalities at operation. JJG Hoorweg-Nijman, Department of Paediatrics, Free University Hospital. PO Box 7057, 1007 MB Amsterdam, The Netherlands


PEDIATRICS ◽  
1968 ◽  
Vol 41 (2) ◽  
pp. 421-427
Author(s):  
Vernon C. Stevens ◽  
Joseph W. Goldzieher

Four normal children and eight subjects with congenital adrenal hyperplasia (ranging in age from 4 to 29 years) were studied for their urinary excretion of steroids, follicle stimulating hormone (FSH), and luteinizing hormone (LH) under control conditions and during suppression with corticosteroids. In the normal children, urinary FSH and LH excretion was less than 0.5 and 0.24 I.U. per day, respectively, whereas in four of five children with congenital adrenal hyperplasia they were detectable and often of adult magnitude. Gonadotropin levels in the adult patients were more variable. Suppression of steroid output by corticosteroid therapy caused a fall of FSH in three of six patients. LH excretion was unchanged in five and rose in two. These findings suggest that the compensatory pituitary hyperactivity in congenital adrenal hyperplasia is not limited to the pituitary-adrenal mechanism but has repercussions in gonadotropin regulation as well.


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