caa a22 4500 46580537X CHVBK 20180323112102.0 cr unu---uuuuu 170327e19900501xx s 000 0 eng 10.1007/BF03350681 doi (NATIONALLICENCE)springer-10.1007/BF03350681 Hypothalamic-pituitary-testicular function in 70 patients with myotonic dystrophy [Elektronische Daten] [J. Vazquez, J. Pinies, P. Martul, A. De los Rios, S. Gatzambide, M. Busturia] Hypothalamic-pituitary-testicular function was studied in 70 patients with myotonic dystrophy (MD). The diagnosis was confirmed by electromyography. The mean age of the patients was 36.2 ± 13.2 yr and the duration of the disease was 11.17 ± 8.01 yr. Testicular atrophy (testes ≤ 12 ml on a Prader orchidometer) was present in 65.5% of patients. Fertility among married patients was 66.6%. Mean testosterone plasma levels were 438 ± 298 ng/dl vs 520 ± 185 ng/dl in the control group (P = NS). Basal plasma FSH and LH levels, and their response after the administration of 100 mcg of LH-RH were significantly increased although a wide dispersion was observed. Sperm count was carried out in 27 cases, showing a normal count in 7, oligospermia in 12, and azoospermia in 8 patiens. Testicular biopsy was performed in 45 patients being normal in 2, showing mild testicular damage in 8, moderate in 14, and severe in 18; it was nule in 3 of them. A significant relationship between testicular atrophy and the sperm count (p < 0.01), testicular damage and testicular atrophy (p < 0.025), and sperm count and testicular damage (p = 0.017) was found. Basal plasma FSH and LH level were significantly related to the degree of damage in the testicular biopsy. All these findings indicate a primary testicular pathology, prevailing tubular over interstitial damage. We have not found any association between the duration of the disease and gonadal dysfunction. Italian Society of Endocrinology (SIE), 1990 Myotonic dystrophy nationallicence hypogonadism nationallicence hypothalamic-pituitary-testicular function nationallicence sperm count nationallicence testicular damage nationallicence Vazquez J. Servicio de Endocrinologia, Hospital de Cruces, Vizcaya, Spain aut Pinies J. Servicio de Endocrinologia, Hospital de Cruces, Vizcaya, Spain aut Martul P. Servicio de Endocrinologia, Hospital de Cruces, Vizcaya, Spain aut De los Rios A. Servicio de Anatomia Patológica, Hospital de Cruces, Vizcaya, Spain aut Gatzambide S. Servicio de Endocrinologia, Hospital de Cruces, Vizcaya, Spain aut Busturia M. Servicio de Endocrinologia, Hospital de Cruces, Vizcaya, Spain aut Journal of Endocrinological Investigation Springer International Publishing 13/5(1990-05-01), 375-379 0391-4097 13:5<375 1990 13 40618 https://doi.org/10.1007/BF03350681 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF03350681 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Vazquez J. Servicio de Endocrinologia, Hospital de Cruces, Vizcaya, Spain aut NATIONALLICENCE 700 1- Pinies J. Servicio de Endocrinologia, Hospital de Cruces, Vizcaya, Spain aut NATIONALLICENCE 700 1- Martul P. Servicio de Endocrinologia, Hospital de Cruces, Vizcaya, Spain aut NATIONALLICENCE 700 1- De los Rios A. Servicio de Anatomia Patológica, Hospital de Cruces, Vizcaya, Spain aut NATIONALLICENCE 700 1- Gatzambide S. Servicio de Endocrinologia, Hospital de Cruces, Vizcaya, Spain aut NATIONALLICENCE 700 1- Busturia M. Servicio de Endocrinologia, Hospital de Cruces, Vizcaya, Spain aut NATIONALLICENCE 773 0- Journal of Endocrinological Investigation Springer International Publishing 13/5(1990-05-01), 375-379 0391-4097 13:5<375 1990 13 40618 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer