Linear and Nonlinear Heart Rate Variability Analysis in Gonadal Dysgenesis (Swyer Syndrome): A Case Report

Abstract Swyer syndrome is one of the disorders of sexual differentiation. Previous studies have demonstrated increased sympathetic activity with heart rate variability (HRV) analysis with decreasing estradiol levels. One patient presented a pure 46, XY gonadal dysgenesis with female phenotype. Cardiac autonomic modulation was assessed through HRV analysis while at rest. This research analyzed linear and nonlinear indexes. HRV analysis showed reduced parasympathetic and global modulation with an apparent increase in sympathetic tone and a loss of HR fractal dynamics toward correlated behavior, characterized by low entropy and high determinism of time series.

Effect of pinealectomy and gonadectomy on dopaminergic neurons of mesencephalon in Parkinson's disease model rats / 中华行为医学与脑科学杂志

Objective To investigate the effect of PX and GDX on dopaminergic neurons of mesencephalon in Parkinson's disease model rats.Methods Eighty male Wistar rats were randomly divided into five groups:NS,N+6-OHDA,PX+6-OHDA,GDX+6-OHDA and PX+GDX+6-OHDA group;16 rats in each group.60 days after operation,6-OHDA was injected into SNc and VTA in the right side of the mesencephalon.Mter 14 days,the rats were sacrificed.HE staining was used to observe the proliferation of glial cells.The number of astrocytes was observed by GFAP staining.The number of apoptotic cells was observed by TUNEL staining.Immunohistochemical staining (S-P Method) was used to detect the changes of TH-positive cells and fiber,apoptosis-related proteins Bax and Bcl2.Results (1) The numbers of proliferated glial cells in SNc and VTA were significantly increased according to the following sequence:NS,N+6-OHDA,PX + 6-OHDA,GDX+ 6-OHDA,and PX+GDX+6-OHDA (HE staining:Hc =280.178,P＜0.01;Hc =334.260,P＜0.01) (GFAP staining:Hc=367.081,P＜0.01;Hc=376.049,P＜0.01).(2) The numbers of apoptotic cells in SNc and VTA were significantly increased according to the following sequence:NS,N+ 6-OHDA,PX+ 6-OHDA,GDX+6-OHDA,and PX+GDX+6-OHDA (Hc =344.401,P＜0.01;Hc =326.198,P＜0.01).(3) The numbers of TH (+) cells in SNc and VTA were significantly decreased according to the following sequence:NS,N+6-OHDA,PX+ 6-OHDA,GDX+ 6-OHDA,and PX+GDX+ 6-OHDA (Hc =366.532,P＜ 0.01;Hc =372.565,P＜0.01).(4) The gray values of TH(+) positive cells and fiber in SNc and VTA were significantly increased according to the following sequence:NS,N+6-OHDA,PX+6-OHDA,GDX+6-OHDA and PX+GDX+ 6-OHDA (F=517.239,P＜ 0.01;F=526.758,P＜ 0.01).(5) Bax expression:weakly positive in NS group,positive in N+6-OHDA group,and strongly positive in remaining three groups;Bcl2 expression:weakly positive in each group.Conclusion PX and GDX significantly aggravate neurotoxicity of 6-OHDA.And GDX is greater than PX,while PX + GDX is strongest.

Effect of pinealectomy and gonadectomy on dopaminergic neurons of mesencephalon in Parkinson's disease model rats / 中华行为医学与脑科学杂志

Objective To investigate the effect of PX and GDX on dopaminergic neurons of mesencephalon in Parkinson's disease model rats.Methods Eighty male Wistar rats were randomly divided into five groups:NS,N+6-OHDA,PX+6-OHDA,GDX+6-OHDA and PX+GDX+6-OHDA group;16 rats in each group.60 days after operation,6-OHDA was injected into SNc and VTA in the right side of the mesencephalon.Mter 14 days,the rats were sacrificed.HE staining was used to observe the proliferation of glial cells.The number of astrocytes was observed by GFAP staining.The number of apoptotic cells was observed by TUNEL staining.Immunohistochemical staining (S-P Method) was used to detect the changes of TH-positive cells and fiber,apoptosis-related proteins Bax and Bcl2.Results (1) The numbers of proliferated glial cells in SNc and VTA were significantly increased according to the following sequence:NS,N+6-OHDA,PX + 6-OHDA,GDX+ 6-OHDA,and PX+GDX+6-OHDA (HE staining:Hc =280.178,P＜0.01;Hc =334.260,P＜0.01) (GFAP staining:Hc=367.081,P＜0.01;Hc=376.049,P＜0.01).(2) The numbers of apoptotic cells in SNc and VTA were significantly increased according to the following sequence:NS,N+ 6-OHDA,PX+ 6-OHDA,GDX+6-OHDA,and PX+GDX+6-OHDA (Hc =344.401,P＜0.01;Hc =326.198,P＜0.01).(3) The numbers of TH (+) cells in SNc and VTA were significantly decreased according to the following sequence:NS,N+6-OHDA,PX+ 6-OHDA,GDX+ 6-OHDA,and PX+GDX+ 6-OHDA (Hc =366.532,P＜ 0.01;Hc =372.565,P＜0.01).(4) The gray values of TH(+) positive cells and fiber in SNc and VTA were significantly increased according to the following sequence:NS,N+6-OHDA,PX+6-OHDA,GDX+6-OHDA and PX+GDX+ 6-OHDA (F=517.239,P＜ 0.01;F=526.758,P＜ 0.01).(5) Bax expression:weakly positive in NS group,positive in N+6-OHDA group,and strongly positive in remaining three groups;Bcl2 expression:weakly positive in each group.Conclusion PX and GDX significantly aggravate neurotoxicity of 6-OHDA.And GDX is greater than PX,while PX + GDX is strongest.

Deficiency of sex hormones does not affect 17-ß-estradiol-induced coronary vasodilation in the isolated rat heart

The relaxation of coronary arteries by estrogens in the coronary vascular beds of naive and hypertensive rats has been well described. However, little is known about this action in gonadectomized rats. We investigated the effect of 17-ß-estradiol (E2) in coronary arteries from gonadectomized rats, as well as the contributions of endothelium-derived factors and potassium channels. Eight-week-old female and male Wistar rats weighing 220-300 g were divided into sham-operated and gonadectomized groups (n=9−12 animals per group). The baseline coronary perfusion pressure (CPP) was determined, and the vasoactive effects of 10 μM E2 were assessed by bolus administration before and after endothelium denudation or by perfusion with NG-nitro-L-arginine methyl ester (L-NAME), indomethacin, clotrimazole, L-NAME plus indomethacin, L-NAME plus clotrimazole or tetraethylammonium (TEA). The CPP differed significantly between the female and sham-operated male animals. Gonadectomy reduced the CPP only in female rats. Differences in E2-induced relaxation were observed between the female and male animals, but male castration did not alter this response. For both sexes, the relaxation response to E2 was, at least partly, endothelium-dependent. The response to E2 was reduced only in the sham-operated female rats treated with L-NAME. However, in the presence of indomethacin, clotrimazole, L-NAME plus indomethacin or L-NAME plus clotrimazole, or TEA, the E2 response was significantly reduced in all groups. These results highlight the importance of prostacyclin, endothelium-derived hyperpolarizing factor, and potassium channels in the relaxation response of coronary arteries to E2 in all groups, whereas nitric oxide may have had an important role only in the sham-operated female group.

Hippocampal serotonin-2A receptor-immunoreactive neurons density increases after testosterone therapy in the gonadectomized male mice / 대한해부학회지

The change of steroid levels may also exert different modulatory effects on the number and class of serotonin receptors present in the plasma membrane. The effects of chronic treatment of testosterone for anxiety were examined and expression of 5-HT(2A) serotonergic receptor, neuron, astrocyte, and dark neuron density in the hippocampus of gonadectomized male mice was determined. Thirty-six adult male NMRI mice were randomly divided into six groups: intact-no testosterone treatment (No T), gonadectomy (GDX)-No T, GDX-Vehicle, GDX-6.25 mg/kg testosterone (T), GDX-12.5 mg/kg T, and GDX-25 mg/kg T. Anxiety-related behavior was evaluated using elevated plus maze apparatus. The animals were anesthetized after 48 hours after behavioral testing, and decapitated and micron slices were prepared for immunohistochemical as well as histopathological assessment. Subcutaneous injection of testosterone (25 mg/kg) may induce anxiogenic-like behavior in male mice. In addition, immunohistochemical data reveal reduced expression of 5-HT(2A) serotonergic receptor after gonadectomy in all areas of the hippocampus. However, treatment with testosterone could increase the mean number of dark neurons as well as immunoreactive neurons in CA1 and CA3 area, dose dependently. The density of 5-HT(2A) receptor-immunoreactive neurons may play a crucial role in the induction of anxiety like behavior. As reduction in such receptor expression have shown to significantly enhance anxiety behaviors. However, replacement of testosterone dose dependently enhances the number of 5-HT(2A) receptor-immunoreactive neurons and interestingly also reduced anxiety like behaviors.

Tumores germinales gonadales en niños: experiencia de 20 años en un centro de referencia pediátrico / Gonadal germ cell tumors in children: experience of 20 years in a pediatric referral center

Antecedentes. Los tumores de células germinales son un grupo heterogéneo de neoplasias que corresponden al 1-3% de los tumores en pediatría. Por lo general, se manifiestan clínicamente con masa testicular o dolor abdominal. Objetivo. Describir las características de los pacientes con tumores germinales gonadales en un centro de referencia de cáncer pediátrico en Colombia. Materiales y métodos. Este artículo hace una revisión retrospectiva recopilando 20 años de experiencia en el diagnóstico y manejo de estos tumores en la Fundación Hospital de la Misericordia. Resultados. Se encontraron 79 pacientes, 35 hombres y 44 mujeres, con un promedio de edad, al diagnóstico, de 6 años. El teratoma fue la neoplasia más frecuente en el sexo femenino y el tumor del seno endodérmico en el masculino. El 70,9% de los pacientes se diagnosticaron en estadio I; se tienen datos de marcadores tumorales en 84,8%. El 98,7% se manejaron con cirugía y 58,2% recibieron además quimioterapia, especialmente protocolo BEP. 96% alcanzaron remisión de la enfermedad, 3 pacientes fallecieron. Conclusiones. El estudio demuestra buenos resultados en el manejo de los tumores germinales gonadales con el protocolo establecido de manejo quirúrgico y quimioterapia, se encontró una baja tasa de recurrencia en el periodo evaluado y alto porcentaje de niños libres de enfermedad.

Background. Germ cell tumors are a heterogeneous group of neoplasms corresponding to 1-3% of pediatric tumors. They usually manifest clinically as testicular mass or abdominal pain. Objective. To make a description of gonadal germ cell tumor types in pediatric referral center in Colombia. Materials and methods. This article takes a retrospective review compiling 20 years of experience in diagnosis and management of these tumors in the Fundación Hospital de La Misericordia. Results. We found 79 patients, 35 men and 44 women, with an average age at diagnosis of 6 years. The teratoma was the most common neoplasia in females and endodermal sinus tumor in men. 70.9% of patients were diagnosed with stage I. In 84,8% measuring tumor markers were obtained. 98.7% were managed with surgery and 58.2% also received chemotherapy, especially BEP protocol. 96% achieved disease remission, 3 patients died. Conclusions. The study shows good results in the management of gonadal germ cell tumors with the established protocol and low recurrence rate in the evaluated period and high rates of children free of disease, were found a low rate of recurrence in the assessment period and high percentage of free of disease.

Gonadectomia pré-puberal em cães e gatos / Prepubertal gonadectomy in dogs and cats

A população de cães e gatos errantes é um problema grave de saúde pública e bem estar animal e a gonadectomia pré-puberal a partir de seis semanas de idade é a base para controle populacional efetivo. Os efeitos benéficos e maléficos são discutidos nesta revisão, auxiliando o médico veterinário a obter conhecimento e fundamentação científica para análise crítica do tema, propagação dessa prática e conscientização de proprietários.

Stray dogs and cats is a serious problem to public health and animal welfare. Prepubertal gonadectomy from six weeks of age is a solution to effective pet overpopulation control. The benefits and adverse effects of this procedure are discussed in this paper, allowing the veterinarians to obtain knowledge and scientific basis to critical analysis to the topic, practice spread and owner awareness.

A large advanced seminoma in an older woman with androgen insensitivity syndrome / 대한생식의학회지

A 58-year-old woman who presented with inguinal hernia for the first time was diagnosed as seminoma and complete androgen insensitivity syndrome (CAIS). The patient received a late diagnosis, and therefore she could not take a proper management. CAIS is a rare X-linked recessive disease with an XY karyotype that is caused by androgen receptor defects. It usually present with primary amenorrhea or inguinal hernia. The risk of malignant transformation of undescended testis increases with age, thus gonadectomy should be performed after puberty. We present a case of large advanced seminoma in a woman with CAIS who was neglected and diagnosed lately.

Gonadectomía bilateral e histerosgalpingectomía laparoscópica en un adolescente con desorden de desarrollo sexual ovotesticular: reporte de caso / Bilateral gonadectomy and laparoscopic hysterosalpingectomy in an adolescent with ovotesticular disorder of sexual development: case Report

Los desórdenes de desarrollo sexual hacen referencia a niños cuyos genitales al nacer presentan características de ambos sexos o aquellos que, teniendo un fenotipo normal, presentan alteraciones del desarrollo en la pubertad o adolescencia. Estos desórdenes representan un reto diagnóstico y terapéutico, debido a que pueden ser desencadenados por diferentes entidades nosológicas, y por otro lado, pacientes portadores de las mismas patologías pueden presentar diferentes alteraciones anatómicas. La laparoscopia juega un importante papel en el abordaje diagnóstico de estos pacientes; permite la visualización de los genitales internos y la toma de muestras de tejidos para estudios histológicos. Desde el punto de vista terapéutico, la cirugía laparoscópica permite la extirpación de estructuras internas opuestas al sexo definitivo del paciente. Se reporta el caso de un adolescente con desorden del desarrollo sexual ovotesticular (Hermafrodita verdadero) a quien una vez definido el sexo social como varón, se le extirparon a través del abordaje laparoscópico, los remanentes embrionarios derivados del Müller. Se practicó mamoplastia reductora e implantación de prótesis testiculares a través de cirugía convencional.

Disorders of sexual development in adolescents refer to children whose genitals at birth present characteristics of both genders, or to those children with normal phenotype that present a development alteration during puberty or adolescence. These disorders represent diagnostic and therapeutic challenges, because they can be triggered by different nosological entities; and on the other hand, patients carrying the same pathologies can present different anatomical alterations. Laparoscopy plays an important role in the diagnostic approach of these patients because, besides the fact that it allows an excellent visualization of the internal genitals anatomy, it also allows tissue sample taking for histological studies. On the other hand, from a therapeutic point of view, laparoscopic surgery allows removal of internal structures opposed to the patient’s definite gender. We report the case of an adolescent with ovotesticular disorder of sexual development (True Hermaphrodite) from whom, once his male gender was socially defined, the embrionary remnants derived from Müller were removed through laparoscopic approach. Mamoplastic reduction and testicular prosthesis implantation were also practiced through conventional surgery.

Effects of gender and gonadectomy on growth and plasma cholesterol levels in pigs

We conducted two studies to determine the effect of gender, gonadectomy (GDX) on growth and plasma cholesterol levels in pigs. In experiment 1, five sham-operated and five GDX female Landrace pigs (26 kg) were allowed to have free access to water and feed up to market weight (approximately 100 kg). Body weight and feed consumption were recorded biweekly, and daily body weight gain, daily feed intake and feed efficiency (gain/feed) were calculated during the feeding period. In experiment 2, 10 male (26 kg) and 10 female (26 kg) Landrace pigs were used; five male and five female pigs were assigned to sham-operated or GDX. Pigs were allowed to have free access to water and a diet without added cholesterol (Table 1) until they were 6 months old (male 104 and female 98 kg) and thereafter they were fed a hypercholesterolemic diet (Table 1) containing 0.5% cholesterol and 0.1% cholate for 10 days. GDX of female pigs increased average daily gain (P0.05) on feed efficiency. Plasma cholesterol levels in pigs fed a hypercholesterolemic diet for 10 days were much higher (P<0.05) in females than in males (161 vs 104 mg/100 mL plasma), and were increased by GDX only in male pigs. HDL-cholesterol/LDL+VLDL-cholesterol ratio appeared to be higher in males than in females, and was not influenced by GDX in either sex. Results suggested that the lower growth rate of female pigs than their male counterparts is attributable to the ovarian activity, and the lower plasma cholesterol level in male than in female pigs fed a hypercholesterolemic diet is due to the testicular activity.

45,X / 47,XYY Mosaic Turner Syndrome / 대한산부인과학회잡지

45,X/47,XYY mosaicism is a rare sex chromosomal disorder with clinical information limited to 25 cases in the literature. We report an unusual mosaic Turner syndrome case in a 35-year old Korean woman with a phenotypic female, primary amenorrhea, short stature, immature secondary sexual characteristics. Cytogenetic analysis including G- and Q-banding revealed 45,X/47,XYY mosaicism, and SRY gene was demonstrated by polymerase chain reaction(PCR). Prophylactic bilateral gonadectomy was performed because the presence of Y-chromosomal sequences in Turner stigmata may predispose this patient to gonadoblastoma formation.

Pelviscopic Gonadectomy in two cases of Complete Androgen Insensitivity Syndrome / 대한산부인과학회잡지

Androgen insensitivity syndrome is a genetic syndrome characterized by complete or partial resistance of end organs to the peripheral effect of androgen. The phenotype of this condition is female, despite the normal male karyotype 46,XY. This is the third most common cause of primary amenorrhea after gonadal dysgenesis and mullerian agenesis. There is a congenital insensitivity to androgens, transmitted by means of a maternal X-linked recessive gene responsible for the androgen intracellular receptor. Prophylactic gonadectomy in usually performed due to increased risk for development of malignancy and possible virilization. Pelviscopy provides a minimally invasive technique for the accurate diagnosis and also provides the opportunity for therapeutic management of these patients. Recently we experienced two cases of this syndrome and removed the gonad by pelviscopic surgery, so we presented them with brief review of literature.

The Effects of Gonadectomy and Prenatal Testosterone Injection on Galanin Immunoreactive Cells in the Medial Preoptic Area of Rats / 대한해부학회지

Within the medial preoptic area[MPOA], several cytoarchitectonically defined cell groups are sexually dimorphic in their morphology. Specially, the sexual dimorphic nucleus of the preoptic area[SDN-POA] is reported an example of a morphological sex difference in the rat hypothalamus which is influenced by gonadal steroid hormones. Thus, we detemined the distribution of Galanin-immunoreactive[Gal-I] cells and fibers within MPOA and their morphological response to gonadal steroids which is influenced by gonadectomy or prenatal restosterone treatments were observed. The Gal-I cells were appeared within the medial preoptic area. In the males, the volume and number of Gal-I nerve cell bodies were greater than that of females. But the female which treated prenatal testosterone injection had many Gal-I neurons than infact female. And the males that decreased the volume of gonadal hormone by gonadectomy were decreased the volume and number of Gal-I neurons than that of normal males. These results suggest that galaninergic cells within the medial preoptic area are influenced by gonadal steroid hormone[testosterone] in the regulation of sexually dimorphic function.

Gonadoblastoma and dysgerminoma associated with 46,XY pure gonadal dysgenesis--a case report

Gonadoblastoma and dysgerminoma developed in a 24-year-old phenotypic female patient with 46,XY pure gonadal dysgenesis. This patient presented with primary amenorrhea. Clinical characteristics showed a typical stigmata of gonadal dysgenesis: primary amenorrhea, sexual infantilism, a small uterus and bilateral streak gonads. A 46,XY karyotype was made by lymphocyte culture. The patient was counseled to undergo a prophylactic bilateral gonadectomy, but she refused. Three years and three months after the initial diagnosis she felt a growing pelvic mass. Bilateral gonadectomy and total hysterectomy were performed. Histological examination revealed gonadoblastoma and dysgerminoma on both gonads. After surgery the patient received radiation therapy and also was started on hormone replacement therapy. Two years and two months after treatment by surgery the patient is well and free of recurrence.