Résumé
ABSTRACT We report a 27 -year-old male referred because of hypergonadotropic hypogonadism with low testosterone and azoospermia. At 23 years of age, he underwent an excision of a hypoechoic 0.7 cm nodule of the left testicle. The pathological diagnosis was a Leydig cell tumor. In the right testicle, there were three nodules at ultrasound, the biggest measuring 0.6 cm. Four years later, the nodules in the right testicle were still present and the larger nodule was excised. The biopsy showed tubules with only Sertoli cells in the perinodular zone. Diffuse and nodular hyperplasia of the Leydig cells was found in the interstitium. The pathological diagnosis was Sertoli syndrome with severe hyperplasia of the Leydig cells. With testosterone therapy, LH decreased, and the nodules disappeared. Thereafter, upon interrupting therapy, LH increased, and the nodules reappeared in two occasions. Resuming testosterone treatment, the nodules disappeared again, suggesting a Leydig cell hyperplasia dependent on chronic LH stimulation.
Presentamos un varón de 27 años referido por hipogonadismo hipergonadotrófico con testosterona baja y azoospermia. El paciente tenía el antecedente de un nódulo sólido hipoecogénico de 0,7 cm en el testículo izquierdo, extirpado los 23 años de edad en el año 2002 y diagnosticado patológicamente como tumor de células de Leydig. En ese año se encontraron tres nódulos en el testículo derecho por ultrasonografía, el mayor de 0,6 cm. Cuatro años después, en 2007, los micronódulos del testículo derecho seguían presentes. El mayor de ellos fue extirpado. En la biopsia, había túbulos con solo células de Sertoli en la zona perinodular. En el intersticio había hiperplasia difusa y nodular de las células de Leydig. El diagnóstico patológico fue un síndrome de Sertoli con severa hiperplasia de células de Leydig. La terapia con testosterona disminuyó la LH y los nódulos inesperadamente desaparecieron. En dos ocasiones, al interrumpir esta terapia, la LH aumentó y los nódulos reaparecieron. Este proceso revirtió nuevamente con el uso de testosterona, sugiriendo una hiperplasia de células de Leydig dependiente del estímulo crónico de LH.
Sujets)
Humains , Mâle , Adulte , Testostérone/usage thérapeutique , Testostérone/pharmacologie , Hypogonadisme/anatomopathologie , Hypogonadisme/traitement médicamenteux , Cellules de Sertoli/anatomopathologie , Hyperplasie/anatomopathologie , Cellules de Leydig/anatomopathologieRésumé
Históricamente la sociedad ha rechazado el abuso sexual de menores de 13 años, dictándose leyes al respecto. La justicia luego de un debido proceso condenaba al victimario con reclusión incluso hasta la década del 70-80, con orquiectomía. Los adelantos en neurobiología, endocrinología, sicofarmacología y sicología se consideraron las bases para tratar al pedófilo y someterlo a libertad condicional, ahorrándose el costo financiero de la reclusión de por vida. Diversos países dictaron leyes contra la conducta pedófila. En dicha legislación ejerció gran influencia la promulgación en EE.UU. (estado de Washington "sobre el ofensor sexual" y el dictamen de la Corte Suprema en 1997 en el juicio de Kansas vs Hendricks). En Chile en los 90 el caso del pedófilo apodado "Zacarach" sacó a la luz pública el tema que no se quería ver. En esa fecha se presentó al parlamento un proyecto de Ley para "curar" la pedofilia con acetato de Medroxiprogesterona imitando legislación de EE.UU. Causó sorpresa en el medio endocrinológico que se usara terapia hormonal como "cura" de la pedofilia. Se ha utilizado en varios países la castración química producida por gestágenos o agonístas del GnRH más antiandrógenos (acetato de Ciproterona), para inhibir la secreción y acción de la testosterona disminuyendo líbido y erección. No se ha demostrado que exista curación de la orientación pedófila y existen dudas de la prevención primaria y secundaria de la pedofilia. Pese al adelanto tecnológico en neurociencias para estudio de las zonas vinculadas a la sexualidad, aún no existen marcadores que permitan diagnosticar o pronosticar futuros resultados de la terapia. El tratamiento médico de la pedofilia no garantiza curación ni prevención del delito pedofílico.
Historically, society has rejected sexual abuse of children under 13, with there having been laws enacted in this regard. The judicial system, after a due process, condemned the perpetrator with reclusion and even up until the decades of the 70s and 80s with orchiectomy. Advances in neurobiology, endocrinology, psychopharmacology and psychology were considered the basis for treating the pedophile and putting them on probation, saving the financial cost of imprisonment for life. Multiple countries have enacted laws against pedophilic behaviour. Such legislation was greatly influenced by the enactment in the USA (state of Washington "on the sex offender" and the ruling of the Supreme Court in 1997 in the trial of Kansas against Hendricks). In Chile in the 90s, the case of a pedophile nicknamed "Zacarach" brought to light an issue that nobody wanted to see. Around that time, a bill was presented to Parliament to try and "cure" pedophilia with Medroxyprogesterone acetate, imitating US legislation. It was a surprise in the endocrinological world that hormonal therapy would be used as a "cure" for pedophilia. Chemical castration produced by gestagens or GnRH agonists plus antiandrogens (Cyproterone acetate) has been used in several countries to inhibit the secretion and action of testosterone, reducing libido and erection. It has not been proven that there is a cure for pedophile orientation and there are doubts about the primary and secondary prevention of pedophilia. Despite technological advances in neurosciences for the study of the zones pertaining to sexuality, there are still no indicators that allow for diagnosis or prediction of future results of therapy. The medical treatment of pedophilia does not guarantee cure or prevention of pedophilic crime.
Sujets)
Humains , Mâle , Pédophilie/traitement médicamenteux , Castration/méthodes , Antagonistes des androgènes/usage thérapeutique , Pédophilie/diagnostic , Pédophilie/étiologie , Pédophilie/thérapie , Infractions sexuelles/législation et jurisprudence , Testicule/effets des médicaments et des substances chimiques , Hormone de libération des gonadotrophines/agonistes , Acétate de médroxyprogestérone/usage thérapeutique , Acétate de cyprotérone/usage thérapeutiqueRésumé
Endocrinology step of transgender readjustment therapy is made according to previously published in the part 1 of article: gender identity disorder in rev. chil. endocrinol. diabetes 2015, 8 (4): 167-173.During started puberty in Tanner stage 2-3, the persistence of the experience that their identity male or female gender is not coherent with its bodily, authorize to start the endocrinological therapy, as an important step of body readjusting. In the process of transition from male to female or female to male, should stop pubertal development, what we do with GNRH analogues: intramuscle leuprolideor triptorelin 11.25 mg. every 12 weeks or with medroxyprogesterone acetate 150 mg. monthly. This process continues until 16 years, adding antiandrogen, preferably spironolactone in the process of body readjusting of male to female. At 16 years old, starts the cross hormonal therapy to masculinizing or feminizing. Maintaining gonadotrophin suppression, female to male, testosterone undecanoate or other injectable testosterone esters is administered, customizing the date of administration and inMale to female, daily use of oral estradiol valerate or transdermal gel. Plasma levels of estradiol andtestosterone should not be located in high or supraphysiological range to avoid thromboembolism or polycythemia risk in those who receive testosterone. Should to be explained the time to obtain the bodily effects, achieving a realistic attitude of the goals and the need for regular checks. Attendance to emotional changes, mainly to meet the social gender role. The laboratory, metabolic, hormonal, hemogram and electrolytic changes are evaluated. To be indicated bone densitometry and study images of internal genitals and breasts are necesary...
Sujets)
Humains , Mâle , Adolescent , Adulte , Femelle , Enfant , Hormones/usage thérapeutique , Soins postopératoires , Transsexualisme/traitement médicamenteux , Troubles sexuels et troubles de l'identité sexuelle/traitement médicamenteux , Chirurgie de changement de sexeRésumé
Gender identity disorders (GID) or transsexuality have been a latent issue in Chile 20 years after the first sex reassignment treatment in 1973. Sexual minority groups have posed the problem and even present a bill for civil sexual change. Since the nineties, the number of consultants due to gender identity problems has increased steadily, including children and adolescents. The lack of medical expertise in the area, requires urgent training programs. The first part of this manuscript will deal with the definition, epidemiology, etiology and role of the endocrinologist in the process of sexual reassignment among patients with gender identity disorders. We review sexual differentiation, brain sexual dimorphism and Sexual Development Disorders (SDD) aiming to understand the neurobiological causes of GID and to perform a better differential diagnosis with Sexual Development Disorders. GID are not a psychiatric disease. However the suffering caused by stigmatization, exclusion andabuse generate emotional problems (gender dysphoria). SDD has a genetic and hormonal basis in most cases. Its clinical expression at birth can cause an erroneous civil sex assignation or a discordant civil sex with the sexual identity of the person when there is a surgical correction. GIS without gender dysphoria was excluded as a mental disease from DSM-V and it will also be excluded from the eleventh version of the international classification of diseases. It will maintained as a condition that should be differentiated from SDD and whose treatment should be financed by health systems.
Sujets)
Humains , Mâle , Femelle , Troubles sexuels et troubles de l'identité sexuelle/étiologie , Troubles sexuels et troubles de l'identité sexuelle/thérapie , Troubles sexuels et troubles de l'identité sexuelle/diagnostic , Troubles sexuels et troubles de l'identité sexuelle/épidémiologieRésumé
Hyperprolactinemic males usually have a hypoactive libido and less commonly, erectile dysfunction and disturbances of orgasm and ejaculation. Hyperprolactinemia alters the balance between neurotransmitters, neuropeptides and hormones involved in libido and erection, affecting dopaminergic tone. An imbalance between dopamine, that stimulates sexual function and serotonin that inhibits it, is generated. In the central nervous system, hyperprolactinemia inhibits centers controlling sexual desire and erection. At the neuroendocrine level, it decreases GnRH, LH and testosterone pulses, resulting in a hypogonadotrophic hypogonadism. Erection is also inhibited peripheral actions of low testosterone and high prolactin levels. There is a disturbance of penile smooth muscle relaxation and of the parasympathetic sacrum-penis reflex arch. In experimental animals, acute hyperprolactinemia hampers the central erection mechanism whereas in chronic conditions, peripheral disturbances also occur. Even correcting low testosterone levels, the adverse effects of hyperprolactinemia on sexual function persist. The use of dopaminergic agonists may achieve normal prolactin and testosterone levels resulting in normal sexual function. Chronic hyperprolactinemia results in progressive deterioration of sexual function and a higher hypothalamic damage that does not respond to clomiphene. In this situation and in the presence of sellar tumors that destroy gonadotrophic cells, there is indication of androgenic replacement maintaining the use of dopaminergic agonists...
Sujets)
Humains , Mâle , Adulte , Troubles sexuels d'origine physiologique/étiologie , Hyperprolactinémie/complications , Hyperprolactinémie/diagnostic , Hyperprolactinémie/traitement médicamenteux , Agonistes de la dopamine/usage thérapeutique , Clomifène/usage thérapeutique , Hyperprolactinémie/physiopathologieRésumé
El hirsutismo es un síntoma del hiperandrogenismo femenino a nivel dermatológico y constituye en sí mismo un problema estético y psicosocial para la mujer. Se analizan las drogas involucradas en el tratamiento, el mecanismo de acción y efectos colaterales. La eficiencia y seguridad de ciproterona, espironolactona y flutamida en 3 grupos de mujeres hirsutas se comparan con los datos de la literatura.
Sujets)
Femelle , Humains , Antagonistes des androgènes , Hypoglycémiants , Hirsutisme/traitement médicamenteux , Antienzymes/usage thérapeutique , Contraceptifs/usage thérapeutique , Finastéride/usage thérapeutique , Hyperandrogénie/traitement médicamenteux , Metformine/usage thérapeutique , /antagonistes et inhibiteurs , Thiazolidinediones/usage thérapeutiqueRésumé
Background: Flutamide is an antiandrogen devoid of other hormonal effects, except for a decrease in the secretion of adrenal androgens such as dehydroepidandrosterone sulphate (DHEA-s) and androstenedione. Aim: To assess the effectiveness of flutamide in the treatment of hirsutism, used as monotherapy or combined with oral contraceptives (OC). Patients and methods: Women with peripheral hirsutism (defined as the presence of normal serum androgen levels and normal ovulatory menstrual cycles) were assigned to receive flutamide alone (500 mg/day) or flutamide plus an OC (ethynylestradiol 0.03 mg and desogestrel 150 µg). Hirsute with hyperandrogenism (polycystic ovary syndrome) were assigned to receive flutamide plus an OC. The degree of hirsutism was assessed using a clinical score (Moncada) at three, six and twelve months of therapy. Results: Twenty five women with peripheral hirsutism received flutamide alone and 18 receive flutamide plus the contraceptive. Eighteen women with polycystic ovary syndrome were studied. At three months, the reduction in hirsutism was 11.2, 15.9 and 24.7 percent in women with peripheral hirsutism receiving flutamide alone or flutamide plus OC and in hyperandrogenic women receiving flutamide plus OC, respectively. At twelve months, the figures were 57.2, 57.3 and 52.5 percent respectively. In hyperandrogenic women, at baseline and three months, serum testosterone levels were 0.96 and 0.42 ng/ml and serum DHEA-s levels were 2,980 and 1,490 ng/ml respectively. No collateral effects of treatment or elevations in serum transaminase levels were observed. Conclusions: Flutamide is effective in the treatment of hirsutism in women with normal or elevated androgen levels. Adding OC did not improve the efficacy of the drug.
Sujets)
Humains , Femelle , Adolescent , Adulte , Antagonistes des androgènes , Flutamide/usage thérapeutique , Hirsutisme/traitement médicamenteux , Androgènes/sang , Contraceptifs oraux combinés/usage thérapeutique , Études de cohortes , Marqueurs biologiques/sangRésumé
Actualmente se acepta que en hombres envejecidos sanos existe un descenso progresivo aunque no universal, de la testosterona total y libre, llegando un 20 a 30 por ciento a rango hipogonádico. El diagnóstico de hipogonadismo asociado a envejecimiento es de exclusión. Se requieren estudios para poder diferenciar si los síntomas compatibles con hipogonadismo se deben a falla testosterónica, envejecimiento o alteración de otros ejes endocrinos.
Sujets)
Humains , Mâle , Adulte d'âge moyen , Vieillissement , Hypogonadisme/diagnostic , Hypogonadisme/épidémiologie , Hypogonadisme/psychologie , Climatère , Comportement sexuel/statistiques et données numériques , Comportement sexuel/physiologie , Comportement sexuel/psychologie , Sexualité/statistiques et données numériques , Sexualité/physiologie , Sexualité/psychologie , Testicule/physiologie , Testostérone/analyse , Testostérone/sangRésumé
Background: Hypothalamic dysfunction is a cause of menstrual disturbances in women, in whom other diseases have been discarded. This condition is characterized by a failure of the GNRH pulse generation system and is associated to psychological and environmental factors. A lack of ovulatory response to the administration of clomiphene can be a sign of bad prognosis in hypothalamic dysfunction. Aim: To report the natural history of patients with hypothalamic dysfunction and a bad or deficient response to the administration of clomiphene. Patients and methods: Fifty patients with hypothalamic dysfunction, that consulted for menstrual disturbances at the age of 15 to 20 years old, were studied. All received clomiphene and 31 had an ovulatory response, 12 had menses without ovulation and 7 did not menstruate. Of these 19 women eleven were interviewed again about their menstrual and reproductive history, after a lapse of 9 to 17 years of loss from follow up. Results: Eight of the eleven women had stressful events during adolescence (going away from family house in 3, starting university studies in 3, migration out of the natal country in one and non competitive physical activity in one). All restarted their menses and eight with active sexual life had spontaneous pregnancies, giving birth from two to five children. Ovulatory cycles were documented in women without active sexual life. Conclusions: In teenagers with hypothalamic dysfunction and menstrual disturbances, a deficient or bad response to clomiphene does not necessarily indicate a bad prognosis in terms of menses or fertility
Sujets)
Humains , Adolescent , Adulte , Femelle , Maladies hypothalamiques/complications , Études de suivi , Résultat thérapeutique , Clomifène , Antécédents gynécologiques et obstétricaux , Contraceptifs oraux , Troubles de la menstruation , Maladies hypothalamiques/traitement médicamenteuxRésumé
Los trastornos menstruales son muy frecuentes en los primeros 5 años de edad ginecológica, evolucionando desde un alto porcentaje de ciclos anovulatorios en el primer año postmenárquica a una mayoría de ciclos ovulatorios a los 6 años de edad ginecológica. Estas irregularidades menstruales pueden corresponder a un proceso de maduración del eje hipotálamo-hipófisis-gonadal, o señalar la aparición de cuadros como el síndrome de ovario poliquístico. Otra gran causa de amenorrea y oligoamenorrea desde la pubertad, es el trastorno funcional hipotalámico, en que excluidos todo otro trastorno endocrino, enfermedad sistémica y cuadro orgánico, una alteración funcional en las neuronas del GNRH produce insuficiencia o disfunción hipotalámica de acuerdo al grado de compromiso de la pulsatilidad del GNRH. Se presenta las características clínicas y evolución de un grupo de pacientes con disfunción hipotalámica diagnosticada en la adolescencia, y reevaluadas en la vida adulta.
Menstrual disturbances are very cycles frecuent during the first 5 years after menarche. High prevalence of anovulatory cycles occur in the first year. In contrast high prevalence of ovulatory cycles occur at 6 years postmenarchal. Menstrual disturbances have been associated with hypothalamic-pituitary-ovarian axis madurative phenomenon or pathology of pubertal root as polycystic ovary syndrome. Hypothalamic dysfunction is other cause of menstrual disturbances when other diseases have been discarded. This condition is caracterized by a alteration of the GNRH pulse generation system. We report the natural history of reproductive and menstrual evolution in women with hypothalamic dysfunction developed in adolescence.
Sujets)
Humains , Mâle , Adolescent , Troubles de la menstruation/physiopathologie , Pronostic , Évolution CliniqueRésumé
Background: Spironolactone has an anti androgenic effect, inhibiting the binding of androgens to their receptor. This antagonistic effect is the basis for the use of spironolactone in the treatment of hirsutism. Aim: To study the effectiveness and safety of spironolactone in the treatment of hirsute women and of the association of spironolactone plus dexamethasone in the treatment of hirsutism with glucocorticoid sensitive hyperandrogenism. Patients and method: Sixteen women (group 1) with peripheral hirsutism (defined as those with normal androgens levels, normal menstrual cycles and ovulation) and 24 women (group 2) with glucocorticoid sensitive hyperandrogenic hirsutism were studied. Group 1 was treated with spironolactone 50 mg bid and group 2 with same spironolactone dose plus dexamethasone 0.5 mg at 23 h during one month and 0.25 mg thereafter. Patients were followed during one year. Results: After one year of treatment, a 54 percent reduction in Moncada hirsutism escore was observed in group 1 and 52 percent reduction in group 2. Observed secondary effects of spironolactone were increases in diuresis, fatigability, acne aggravation and seborrhea in two patients. Two additional patients had spotting. No secondary effect attributable to glucocorticoid use were observed. Conclusions: Spironolactone is effective and safe in the treatment of hirsutism. Androgenic supression did no increases its effectiveness, underscoring the peripheral anti androgenic activity os spironolactone
Sujets)
Humains , Femelle , Adolescent , Adulte , Spironolactone/pharmacologie , Dexaméthasone/pharmacologie , Hirsutisme/traitement médicamenteux , Potassium/sang , Spironolactone/administration et posologie , Spironolactone/effets indésirables , Dexaméthasone/administration et posologie , Dexaméthasone/effets indésirables , Études prospectives , Hyperandrogénie/traitement médicamenteux , Association de médicaments , Hirsutisme/diagnostic , Androgènes/sangRésumé
Background: Oligomenorrhea, defined as a menstrual cycle lasting 36 to 90 days, can be a normal condition in the first years after the menarche. When it persists or appears after a period of normal menstrual cycles, an underlying illness must be sought. Aim: To assess ovulation and causes of anovulatory cycles in women with oligomenorrhea, compared with causes of secondary amenorrhea. Patients and methods: One hundred one women of less the 35 years old, presenting with oligomenorrhea persisting 5 years after menarche or lasting more than two years after a period of normal menstrual cycles, were studied. Ovulation was studied measuring serial plasma progesterone during normal or induced (with intramuscular progesterone) menstrual cycles. Results: Eighty nine percent of women had anovulatory oligomenorrhea. The main causes were polycystic ovarian disease in 51percent and hypothalamic dysfunction in 31percent. Thirty percent of women with secondary amenorrhea had polycystic ovarian disease and 14percent had hyperprolactinemia. Women older than 20 years old or with more than 10 years of gynecological age had a higher frequency of polycystic ovarian disease and a lower prevalence of hypothalamic dysfunction. Conclusions: There is a high frequency of anovulatory oligomenorrheas. Therefore, this symptom deserves a thorough endocrinological assessment to uncover underlying diseases. Special attention must be paid to polycystic ovary syndrome, due to its importance in internal medicine as a risk factor for myocardial infarction, high blood pressure, and type 2 diabetes mellitus
Sujets)
Humains , Femelle , Adolescent , Adulte , Oligoménorrhée/diagnostic , Oligoménorrhée/étiologie , Aménorrhée/diagnostic , Anovulation/physiopathologie , Maladies hypothalamiques/complications , Ovulation/physiologie , Syndrome des ovaires polykystiques/complicationsRésumé
The approach to adrenal hyperandrogenism, due to genetical or non-genetical enzymatic alterations, has changed dramatically during the last 50 years. To allow a better understanding of the subject, we focused it from a historical perspective, defining four stages analyzed in detail. Now there is consent on the existence of a functional adrenal hyperandrogenism, which has an important role in acne, hirsutism and menstrual disturbances
Sujets)
Humains , Femelle , Hyperandrogénie/physiopathologie , Histoire Naturelle des Maladies , Hyperandrogénie/étiologie , Hirsutisme/étiologie , Androstènedione , Hypercorticisme/physiopathologie , Syndrome des ovaires polykystiques/physiopathologie , Troubles de la menstruation/étiologieRésumé
Adrenal androgen hypersecretion either produced by genetic defects or reticular disfunction, is reduced by exogenous glucocorticoid administration and as with any suppression therapy, it should relapse when the therapy is discontinued. However, prolonged remissions of adrenal androgen hypersecretion after discontinuing glucocorticoids have been described. We report 15 patients with adrenal hyperandrogenism and elevated levels of dehydroepiandrosterone sulfate that received treatment with dexamethasone. After 1 month of treatment with dexamethasone 0.5 mg/day, dehydroepiandrosterone sulfate levels returned to normal and remained so during a mean of 19 months receiving dexamethasone 0.25 mg/day. One year after discontinuing therapy, hormone levels continued within normal range in all patients. It is concluded that a long remission period of adrenal hyperandrogenism was achieves after discontinuing glucocorticoid therapy
Sujets)
Humains , Femelle , Adolescent , Adulte , Hyperandrogénie/traitement médicamenteux , Glucocorticoïdes/administration et posologie , Testostérone/sang , Acné juvénile/étiologie , Déhydroépiandrostérone/sang , Hirsutisme/étiologie , Antagonistes des androgènes/administration et posologie , Androstènedione/sang , Troubles de la menstruation/étiologieRésumé
En la anamnesis de la hirsuta, destaca el inicio peripuberal del hirsutismo y en el examen físico la importancia del vello pubiano que aporta el 40 por ciento del score total. Del 74 por ciento de hirsutas hiperandrogénicas, el 34 por ciento poseen elevación exclusiva de la testosterona, el 24 por ciento de la dehidroepiandrosterona sulfato y el 42 por ciento un alza de ambas. El alto porcentaje de dehidroepiandrosterona sulfato aumentada, sola o junto a testosterona, sugiere un compromiso suprarrenal en la etiología del hirsutismo, respaldado por un 50 por ciento de prueba de estimulación suprarrenal con hiperrespuesta de este andrógeno, en hirsutas y en pacientes con síndrome de ovario poliquístico. El alza de la 17 hidroxiprogesterona basal, obliga a realizar prueba de estimulación cob ACTH para descartar su origen ov+arico y confirmar el déficit de la 21 hidroxilasa. Un 5 por ciento de las hirsutas tenían acantosis nigricans con resistencia insulínica y alteraciones lipídicas. Se analiza el tratamiento con antiandrógenos, ciproterona y espironolactona y la frenación con glucocorticoides