[Hypogonadism caused by Gorlin-Goltz syndrome]. / Hipogonadismo causado por el síndrome de Gorlin-Goltz.

The Gorlin-Goltz syndrome is a dominant autosomic disorder characterized by cancerigenic predisposition and multiple development defects, apparently without reproductive compromise. The complex is characterized by four primary symptoms, which include nevoid basal cell epitheliomas malignantly prone, keratocystic jaw, skeletal abnormalities and intracranial calcifications. Apparently, reproductive problems reported had been rarely associated with this syndrome. We present the case of a patient with clinic stigmatae of Gorlin-Goltz syndrome, who had a characteristic progress as seen in the literature; he was the fifth product of a 43 year-old female (father was 48 years old); who at birth disclosed right eye microftalmy, bilateral cryptorchidism surgically treated at age of six. At puberty, an odontogenic cyst of the jaw was noted and enucleated. He also showed facial nevi in neck, thorax and abdomen. When he was admitted being 14 years old in our clinic, he had recurrent bilateral cryptorchidism, sexual immatturity and infertility. It is important to take into consideration Gorlin-Goltz stigmatae in cases of hypogonadism in order to recognize a further genetic influence.

[Present usefulness of diagnostic tests in hyperandrogenism]. / Utilidad actual de las pruebas diagnósticas de hiperandrogenismo.

There are diagnostic tests that allow to differentiate feminine hyperandrogenism of tumorous, non-tumorous, ovarian, adrenal and peripheral origin. We made a review of the current available tests accordingly to a case of amenorrhea and hyperandrogenism. By means of the low-dose dexamethasone suppression and human chorionic gonadotropin hormone (hCG) stimulation test we confirmed a suprarenal participation. There was spontaneous menses when treated with dexamethasone.

[Spermatogenesis arrest]. / Detención de la espermatogénesis.

Spermatogenesis arrest is a complex process of interruption in the differentiation of germinal cells of specific cellular type, which elicits an altered spermatozoa formation. In contrast, hypospermatogenesis is defined as a decrease in number of germ cells and its proportion. Factors identified intervening upon spermatogenesis arrest are: genetic, hormonal, growth factors, interaction between Sertoli and germ cells and ectoplasmic specialization integrity of spermatozoa. In addition, environmental toxic effects have shown to exert subletal and letal cellular damage with gene disruption. Hence in this work we review sperm physiology along with etiologic elements associated to spermatogenesis arrest delineating the most appropriate conduct for diagnosis and treatment.

[Craniopharyngioma and its impact upon human reproduction: analysis of 15 cases at the Hospital Juarez de Mexico]. / Craneofaringioma y sus repercusiones en la reproducción humana: análisis de 15 casos en el Hospital Juárez de México.

BACKGROUND: Craniopharyngiomas are intracranial tumors of non-glial origin derived from cellular remnants of the Rathke's pouch. Their frequency is 1-3% of intracranial tumors and 13% of suprasellar neoplasms. Histologically, it is a benign lesion, albeit clinically severe with a high index of relapse and sequelae upon the reproductive function. OBJECTIVE: To identify the clinical characteristics of patients with craniopharyngiomas before and after treatment. MATERIAL AND METHODS: We reviewed our experience of 15 cases with craniopharyngioma seen at the Hospital Juarez de Mexico from 1995 throughout 2002. RESULTS: Prevalence was higher in males (ratio 2:1) with ages between 6-45 years old (X 17.9 +/- 6.45 years). The most common symptoms were cephalalgia (100%) and visual disorders (93%); the average levels of hormonal determination were in females: FSH 1.0 mIU/mL, LH 0.5 mIU/mL, estradiol 11.0 pg/mL, PRL 80 mg/mL. In males: FSH 1.7 mIU/mL, LH 2.6 mIU/mL, testosterone 0.6 mg/dL and PRL 29 mg/mL. All patients had hypogonadotropic hypogonadism. Tumoral relapse was mostly seen in patients who underwent only surgery (n = 7, 46.6%). The ones with surgery and radiotherapy (n = 8, 53.3%) did not disclose symptoms that required another surgery. CONCLUSIONS: The main repercussion of craniopharyngioma, due to its proximity to hypothalamus and hypophysis, is on the endocrine and reproductive areas of human being.

[Absence of antiosteopenic effect of hormone replacement therapy in postmenopausal women]. / Ausencia de efecto antiosteopénico de la terapia de reemplazo hormonal en mujeres posmenopáusicas.

BACKGROUND: Several studies suggest that hormone replacement therapy (HRT) stops bone loss in postmenopausal women while increasing their bone mineral density up to 20%; however, there are studies where hormone replacement therapy does not increase bone density, only prevents it. OBJECTIVE: To analyze the effect of hormone replacement therapy upon bone mineral density of hip and lumbar spine in postmenopausal women. MATERIAL AND METHODS: Clinical files of several patients with sudden menopause diagnosis seen at the Hospital Juarez of Mexico were reviewed in the year 2000. They received hormone replacement therapy of conjugated estrogens and medroxyprogesterone acetate, with annual controls of estradiol in blood, equal or higher than 50 pg/mL and bone mineral density measurement (BMD) with DEXA (dual energy X-ray absortiometry) central (hip and lumbar spine), and at least one control within a period of four years. 89 patients were included and four groups were determined: all of them had basal densitometry in one (group 1), two (group 2), three (group 3), and four years (group 4) of monitoring. The percentage of bone mineral density was calculated in both anatomic areas and per group. In each study group the number or patients that showed gain, loss or whose mineral bone density stayed the same was assessed. The statistical analysis was made using the Student t test. RESULTS: The 89 patients mean age was of 50.5 (SD +/- 6.42) years. All of them showed spontaneous menopause. The average time of menopause evolution was of 6.9 (SD +/- 4.7) years. The body mass index (BMI) average was of 27.5 (SD +/- 3.97) kg/m2. After admittance, all patients received hormone replacement therapy with conjugated equine estrogens (0.625 mg and medroxyprogesterone acetate 2.5 mg/day). A gain of bone mineral density in the second and third year of treatment in the left hip and in the lumbar spine was observed; however, there were no important differences in any study group when results were treated statistically. CONCLUSION: Hormone replacement therapy was not effective to prevent bone loss, neither to increase bone density. Only one treatment was tested, and might be necessary to investigate the effect of other routes of administration or formulations.

[Infertility due to a pituitary gangliocytoma]. / Gangliocitoma hipofisario como causa de infertilidad.

This tumor of hypophyseal origin is rare and its incidence is estimated from 2-5% of central nervous system neoplasms. It is characterized by enlargement of the sella turcica and by the presence of neuron with adenomatous tissue since it is derived form the neural crest. Clinical symptoms do not differ from pituitary adenomas. The diagnosis is made by histological analysis and prognosis is favorable after surgical excision. It is reported the case of a 27 year old female complaining of abnormal menses who was seen at our clinic due to primary infertility of 6 year duration and galactorrhea. An MRI disclosed a 7 mm lesion within the pituitary area. Prolactin was 167 ng/mL. The patient underwent transphenoidal surgery and the histological report was of gangliocytoma. Two months after surgery the patient resumed menses and 6 months later a MRI did not reveal residual tumor; prolactin levels decreased to 44.8 and 18 ng/mL. Afterwards, the patient became pregnant and had a normal delivery. To our knowledge this is the first report of infertility due to a pituitary gangliocytoma.

[Frequency of altered male factor in an infertility clinic]. / Frecuencia del factor masculino alterado en parejas que consultan por infertilidad.

UNLABELLED: Infertility is the incapacity of a couple to conceive after a year of regular sexual life without using a method for family planning. The infertility state is dependent on the female factor as well as masculine factor; an altered masculine factor is designated when any cause or causes of infertility reside in the male. The masculine factor as a cause of infertility is present in 40 to 50% of cases hence the importance of an integral evaluation of male alterations and its fertility. The World Health Organization (WHO) has proposed to classify the masculine infertility based on semen features. OBJECTIVE: To determine the frequency of the masculine factor altered in association to diagnosed abnormalities, according to the number of affected seminal indexes based on seminograms performed in infertile patients seen at the department of Human Reproductive Medicine, Hospital Juárez de México. MATERIAL AND METHODS: An observational, descriptive, transverse, retrospective study was performed. SUBJECTS: the seminograms practiced were reviewed from 571 clinical files of couples that the consultation for infertility from January 1993 to February 2001. Collection and analysis of semen samples was based on standards settled by WHO. RESULTS: Of 571 seminogram informs, 371 (65%) showed alterations in the seminal indexes, the stocking of age of this group was of 31.89 years +/- SD 6.3 years. Our findings were: azoospermia in 89 (23.98%), astenonecrozoospermia in 44 (11.85%), hypospermia in 43 (11.59%), astenozoospermia in 33 (8.89%), oligoastenozoospermia in 31 (8.35%) hyponecrozoospermia in 30 (8.08%), oligozoospermia in 25 (6.73%), others in 21 (5.7%), necrozoospermia in 19 (5.12%), hypoastenozoospermia in 18 (4.85%), cryptozoospermia in 11 (2.97%), and hypooligoastenozoospermia in 7 (1.88%). In 62.6% of seminograms practiced there was an isolated alteration, in 34.4% two alterations and in the remaining 3% more than two alterations. CONCLUSION: We found masculine factor altered in 65% of couples who were requiring consultation for infertility. The three main alterations of seminal indexes were azoospermia, astenonecrozoospermia and hypospermia.

Preeclampsia-eclampsia: un problema médico diferido / Preeclampsia-eclampsia: a deferred medical problem

La hipertensión arterial asociada a embarazo es un problema de Salud Pública en México por su alto índice de morbilidad y mortalidad tanto para la madre como para el feto. La investigación sobre este problema se ha mantenido diferida por limitaciones en información clínica y epidemiológica, desconocimiento de su etiología así como por su fácil resolución al interrumpirse el embarazo. Se revisan aquí aportaciones hechas al área en nuestro país y conceptos de control y tratamiento

Tumor hipofisario familiar de compromiso somatomamotrópico / Familiar pituitary tumor with somatotropic compromise

Se describe por vez primera el adenoma hipofisario de estirpe familiar, con afección aparente sobre el somatomamotropo responsable de la secreción de hormona de crecimiento y prolactina, cuya transmisión fue de carácter antosómico domiante. Destacó que un par de miembros sin tumor demostrable, cursaron con datos clínicos (fenotipo) de acromegaloidismo. Al investigarse antígenos de histocompatibilidad resaltó que tanto los pacientes con tumor y otros sintomáticos pero sin tumor compartieron los mismos haplotipos, por lo que es muy posible que la investigación de antígenos HLA en los pacientes con tumor hipofisiario ayude a reconocer mejor su naturaleza y frecuencia

Influencia del tumor testicular sobre el eje de hipotálamo, hipófisis y testículo / Testicular tumor influence on the hipothalamus-hypophisis-testicle axis

El teatículo contiene en su interior a las células germinales, que pueden producir cualquier clase de tejido o susbstancia en el organismo. Cuando existe crecimiento celular desorganizado, como en el cáncer, la gónada puede producir substancias, como la gonadotropina coriónica humana (HCG) y la alfafetoproteína (AFP), que se utilizan como marcadores de actividad tumoral. Se determinaron los valores basales de hormona luteinizante (LH), hormona foliculoestimulante (FSH), HGC y AFP e cuatro pacientes con tumor testicular. En tres de ellos el informe histopatológico indicó teratocarcinoma. En estos pacientes los niveles de HGC y AFP se encontraron elevados, en tanto que las gonadotropinas hipofisarias se hallaron bajas: en un paciente con diagnóstico de atrofia testicular todas las determinaciones fueron normales. Se observó que en estos pacientes la HCG circulante inhibió la liberación de LH y FSH.

Síndrome de ovarios poliquísticos: ¿Anomalía hipotalámica o gonadal? / The Syndrome of polycystic ovaries. A hypothalamic or gonadal abnormality?

La etiología del síndrome de ovarios poliquísticos permanece desconocida a pesar de los avances conseguidos por estudios bioquímicos y de morfología. No se ha podido establecer una correlación entre hallazgos histológicos y la producción hormonal hipofisiaria o gonadal; sin embargo, se ha registrado una interacción anormal del eje hipotálamo-hipófisis-gónada, caracterizada por una relación aumentada entre la hormona luteinizante (LH) y la folículo-estimulante (FSH), aunada a sobreproducción de andrógenos, particularmente androstenediona. Las pruebas de reserva hipofisiaria con agonistas de la hormona hipotalámica liberadora de gonadotropinas o GnRH, además de demostrar hiperrespuesta de LH que coincide con elevación de androstenediona, ha permitido apreciar también incremento de los niveles de 17-hidroxiprogesterona. Dado que el patrón de formación de gonadotropinas y hormonas esteroides en la mujer con poliquistosis ovárica es muy semejante al de hombres normales, la hipótesis de que el trastorno básico radica en la conformación de un hipotálamo de tipo masculino, sigue siendo vigente.

Activación de células vivas inmóviles durante la capacitación espermática in vitro / Activation of live nonmotile cells during spermatic capacitating in vitro

Con el objeto de valorar el impacto de la capacitación espermátiza "in vitro" sobre la motilidad de las diferentes poblaciones de espermatozoides de un eyaculado, se analizaron los resultado de este procedimiento en 49 muestras de semen de varones de parejas estériles adscritos al programa de inseminación artificial homóloga del Instituto Nacional de Perinatología. El estudio se llevó a cabo valorando la matilidad de las células de acuerdo a los criterios establecidos por la Organización Mundial de la Salud, antes y después de la capacitación espermática con medio HAM = F10. Se observó disminución en el porcentaje de las células inmóviles (grado 0) de 27.8 ñ 4.6 a 20 ñ 3.2 (X ñ D. E.) después de la capacitación, así como un incremento de la motilidad en la población de espermatozoides más activos (grado 3) de 28.6 ñ 3.9 a 39.1 ñ 3.6 (X ñ D. E.). Ambas diferencias fueron estadísticamente significativas (p < 0.01) y (p < 0.001) respectivamente. Como es de esperarse la motilidad total fue mayor después de la capacitación, con aumento de 70.1 ñ 3.8 (X ñ D. E.) a 78.5 ñ 3.8 (X ñ D.E.). La diferencia en el porcentaje de motilidad pre y postcapacitación en las poblaciones con movimientos grado 1 y 2 no fue significativa. Estos datos sugieren que la capacitación espermática "in vitro" activa en secuencia a todas las poblaciones de gametos del eyaculado