Variante incomum da síndrome de Herlyn- -Werner-Wunderlich: relato de caso / Unsual Herlyn-Werner-Wunderlich syndrome: case report

Malformações müllerianas correspondem a variações anatômicas do trato repro- dutor feminino. Comumente assintomáticas, o diagnóstico e a verdadeira incidên- cia são difíceis de determinar. A síndrome de Herlyn-Werner-Wunderlich, clas- sicamente descrita pela tríade útero didelfo, hemivagina cega e agenesia renal ipsilateral também pode ter variações diferentes. Em virtude da mesma origem embrionária dos tratos genital e urinário, anomalias renais devem ser investigadas nesses casos, sendo a mais comum a agenesia renal. Este artigo relata o caso de uma paciente de 18 anos, do sexo feminino, com história de piocolpo por cinco anos. Em propedêutica complementar, foi identificado útero com septação com- pleta associado a hemissepto de terço superior de vagina, formação de piocolpo e agenesia renal à direita. Apesar de não ser a definição clássica, o quadro está incluído nos casos de síndrome de Herlyn-Werner-Wunderlich.

Mullerian malformations correspond to anatomical variations of the female repro- ductive tract. Commonly asymptomatic, the diagnosis and true incidence are difficult to determine. The Herlyn-Werner-Wunderlich syndrome, classically described by the triad: uterus didelphus, blind hemivagina and ipsilateral renal agenesis, can also have different variations. Due to the same embryonic origin of the genital and urinary tracts, renal anomalies must be investigated in these cases, the most common being renal agenesis. This article reports the case of an 18-year-old female patient with a 5-year history of pyocolpus. In complementary exams, a uterus with complete septa- tion was identified, associated with a hemiseptum in the upper third of the vagina, formation of pyocolpus and renal agenesis on the right side. Despite not being the classic definition, it is included in the cases of Herlyn-Werner-Wunderlich syndrome.

Malformaciones müllerianas: actualización y revisión a propósito de casos clínicos, 2022 / Müllerian malformations: update and review of clinical cases, 2022

Las malformaciones müllerianas (MM) son un grupo de anomalías estructurales originadas por fallas de desarrollo de los conductos paramesonéfricos o de Müller durante las primeras 16 semanas de gestación. Un oportuno diagnóstico y una correcta clasificación permiten ofrecer el mejor manejo y diferenciar aquellas pacientes que requieren tratamiento quirúrgico. Se realizó una revisión de la literatura sobre MM en las bases de datos Epistemonikos, SciELO, Cochrane y PubMed. Se rescataron todas las pacientes ingresadas con diagnóstico de MM. En el año 2021, la American Society of Reproductive Medicine publicó un consenso en el que se estandarizó la nomenclatura, se amplió el espectro y se simplificó la clasificación. La clínica es variada, e incluye pacientes asintomáticas cuyo diagnóstico es un hallazgo por imágenes. Los mejores estudios imagenológicos son la resonancia magnética (RM) y la ultrasonografía 3D, dejando la histeroscopia y la laparoscopia (método de referencia) como procedimiento diagnóstico-terapéutico. Se presentan casos clínicos desarrollados durante el primer trimestre de 2022. Recomendamos la utilización sistemática de la RM para el diagnóstico de anomalías complejas u obstructivas del aparato genital. El tratamiento de estas patologías debe ser realizado por ginecólogos endoscopistas expertos, e incluye tratamiento médico y quirúrgico, el cual debe ser enfocado en cada paciente dependiendo del tipo de MM y de los deseos de fertilidad.

Müllerian malformations (MM) are a group of structural anomalies caused by developmental failure of the paramesonephric or Müllerian ducts during the first 16 weeks of gestation. Timely diagnosis and classification allow us to offer the best management and to differentiate those patients who require surgical treatment. Literature review on MM in Epistemonikos, SciELO, Cochrane and PubMed databases. All patients admitted with a diagnosis of MM were rescued. In 2021, the American Society of Reproductive Medicine publishes a consensus where it standardizes the nomenclature, broadens the spectrum, and simplifies the classification. The clinical picture is varied and includes asymptomatic patients whose diagnosis is an imaging finding. The best imaging studies are magnetic resonance imaging (MRI) and 3D ultrasonography, leaving hysteroscopy and laparoscopy (gold standard) as diagnostic therapeutic. Clinical cases developed during the first trimester 2022 are presented. We recommend the routine use of MRI for the diagnosis of complex and/or obstructive anomalies of the genital tract. The treatment of these pathologies should be performed by expert endoscopic gynecologists and include medical and surgical treatment, which should be focused on each patient, depending on the type of MM and fertility desires.

Estimulación ovárica en síndrome de Mayer-Rokitansky: reporte de caso y revisión de la literatura / Controlled ovarian stimulation in Mayer-Rokitansky syndrome: case report and literature review

Resumen El síndrome de Mayer-Rokitansky-Küster-Hauser (SMRKH) es una anomalía del tracto genital femenino caracterizada por ausencia congénita del útero y porción superior de la vagina. Ocurre en uno de cada 4,500 nacimientos y se diagnostica normalmente durante la adolescencia al presentarse amenorrea primaria. Su función ovárica está preservada, pero la información actual respecto al potencial reproductivo de estas pacientes es limitada. Se presenta el caso de una mujer con diagnóstico de SMRKH sometida a estimulación ovárica para transferencia de embriones a útero subrogado y se discute su potencial reproductivo: técnicas de reproducción asistida, intervenciones e impacto psicológico.

Abstract Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) is a congenital anomaly of the female genital tract characterized by congenital absence of the uterus and upper part of the vagina. It occurs in 4,500 female births and diagnosis is usually made during adolescence when primary amenorrhea presents. They have functioning ovaries but data regarding their reproductive potential is limited. We hereby report the case of a woman diagnosed with MRKH syndrome in whom assisted reproductive techniques were used to try to achieve pregnancy by gestational surrogacy and their reproductive potential is discussed: assisted reproductive techniques, procedures, and psychological impact.

Ovarian reserve in adolescent girls with autoimmune thyroiditis

ABSTRACT Objectives: To assess serum anti-Müllerian hormone (AMH) levels as an ovarian reserve marker in adolescent girls with autoimmune thyroiditis (AIT) and explore the relationship of this marker with autoimmunity and thyroid function biomarkers. Subjects and methods: This study included 96 adolescent girls with newly diagnosed AIT and 96 healthy, age- and sex-matched controls. All participants were evaluated with detailed history taking and physical examination, thyroid ultrasound, and measurement of levels of thyroid-stimulating hormone (TSH), free thyroxin (FT4), free triiodothyronine (FT3), antithyroid peroxidase antibodies (TPOAb), antithyroglobulin antibody (TGAb), estradiol, total testosterone, and anti-Müllerian hormone (AMH) levels. The LH/FSH ratio was also calculated. Among 96 patients evaluated, 78 were overtly hypothyroid and 18 were euthyroid. AMH levels were significantly lower in participants with overt hypothyroidism and euthyroidism compared with controls. Results: Serum levels of AMH correlated negatively with age, body mass index (BMI) standard deviation score (SDS), and TPOAb, TGAb, and TSH levels but positively with FT4 levels. In multivariate analysis, AMH levels correlated significantly with age (odds ratio [OR] = 1.65, 95% confidence interval [CI] 1.18-2.32, p = 0.05), BMI SDS (OR = 2.3, 95% CI, 2.23-3.50, p = 0.01), TSH (OR = 2.43, 95% CI 1.5-2.8, p = 0.01), and TPOAb (OR = 4.1, 95% CI 3.26-8.75, p = 0.001). Conclusions: Ovarian reserve of adolescent girls with AIT, as measured by serum AMH levels, is affected by thyroid autoimmunity and hypothyroidism, indicating a possible need for ovarian reserve monitoring in these patients.

Carcinosarcoma ovárico asociado a carcinoma seroso intraepitelial tubárico

El carcinosarcoma primario ovárico es una neoplasia de baja incidencia, que suele ser diagnosticado en estadios avanzados y cursa con un mal pronóstico. Se comunica el caso de una paciente de 64 años con una tumoración abdominopélvica de 15 cm. El examen histológico evidenció una neoplasia maligna bifásica ovárica asociada a un carcinoma seroso intraepitelial tubárico, hallazgo que estaría en relación con la patogénesis de esta neoplasia.

Primary ovarian carcinosarcoma is a low incidence neoplasm that is usually diagnosed in advanced stages and has a poor prognosis. We report the case of a 64-yearold female patient with a 15 cm abdominopelvic tumor. Histological examination revealed a malignant ovarian biphasic malignancy associated with a serous tubal intraepithelial carcinoma, a finding that would be related to the pathogenesis of this neoplasm.

Mullerian Anomaly with Large Ovarian Mass — Where Two Worlds Collide

When young women diagnosed with Large Ovarian Masses present with associated mullerian abnormality it shakes the world of Gynaecology and requires the joint help of General Gynaecologists and gynae-oncologists as reconstruction of Mullerian anomaly and fertility preservation is as important as management of Malignant Ovarian Masses. We report a case of 21 year, unmarried woman with complaints of primary amenorrhea and large abdominopelvic mass and short, blind vagina. Magnetic Resonance Imaging showed Uterus Didelphis with normal endometrium and upper vaginal agenesis. Computed Tomography images revealed a large ovarian mass. Intraoperatively there was a 30cm large Ovarian Tumour, Didelphyic and hypoplastic (2cm) uterus and 2 cm blind vagina. Management of this case involved extensive discussion among the Gynecologists, Gynae-oncologist and radiologists. In non-oncology setup where the rate of surgeries are high, option between frozen section and Mullerian reconstruction are required especially when the Ovarian mass looks benign as both surgeries require time and expertise. Hence, involvement of patient and family members in decision making form an integral part of management.Intraoperative findings also influence surgical decisions in Mullerian anomaly

Ruptura uterina asociada con útero didelfo y placenta acreta. Reporte de caso / Uterine rupture associated with uterus didelphus and placenta accreta. A case report

Resumen ANTECEDENTES: Las malformaciones müllerianas son consecuencia de una alteración en la formación de los conductos de Müller durante el desarrollo del feto. El momento en que sucede la alteración determina el tipo de malformación. La clasificación actual es la de la American Society for Reproductive Medicine ASMR que se asocia con múltiples complicaciones obstétricas, entre las más graves está la ruptura uterina. CASO CLÍNICO: Paciente primigesta de 23 años, con 39.1 semanas de embarazo, sin antecedentes personales patológicos para el padecimiento actual, sin control prenatal, con dolor abdominal intenso generalizado y disminución de los movimientos fetales desde 12 horas previas a su valoración. Al ingreso de la paciente al hospital su feto se encontró muerto; hemoglobina de 7.9 g/dL, tensión arterial de 96-53 mmHg, taquicárdica, con datos clínicos de irritación peritoneal. En la laparotomía exploradora el feto se encontró muerto, en la cavidad abdominal. Hemoperitoneo de 1300 mL, útero didelfo, con ruptura uterina hacia el fondo. Datos de acretismo placentario. Por lo anterior, se procedió a la histerectomía obstétrica, con sangrado de 2000 cc. Fue necesaria la reanimación y la permanencia de 24 horas en la unidad de cuidados intensivos. La TAC abdomino-pélvica se reportó sin alteraciones renales, con una tumoración adherida cerca del peritoneo parietal sugerente de riñón ectópico. El puerperio trascurrió sin contratiempos por lo que fue dada de alta del hospital. CONCLUSIÓN: En el embarazo, las malformaciones müllerianas son causa de complicaciones obstétricas graves, entre ellas la ruptura uterina. El diagnóstico oportuno es decisivo para la prevención de complicaciones y el control prenatal.

Abstract BACKGROUND: Müllerian malformations are the consequence of an alteration in the formation of the Müllerian ducts during fetal development. The time at which the alteration occurs determines the type of malformation. The current classification is that of the American Society for Reproductive Medicine ASMR, which is associated with multiple obstetric complications, among the most serious of which is uterine rupture. CLINICAL CASE: A 23-year-old primigravid patient, 39.1 weeks pregnant, with no personal pathological history for the current condition, without prenatal control, with severe generalized abdominal pain and decreased fetal movements for 12 hours prior to her evaluation. On the patient admission to the hospital her fetus was found dead; hemoglobin 7.9 g/dL, blood pressure 96-53 mmHg, tachycardic, with clinical data of peritoneal irritation. At exploratory laparotomy the fetus was found dead, in abdominal cavity. Hemoperitoneum of 1300 mL, didelphic uterus, with uterine rupture towards the fundus. Data of placental accretism. Therefore, obstetric hysterectomy was performed, with bleeding of 2000 cc. Resuscitation and a 24-hour stay in the intensive care unit was necessary. The abdomino-pelvic CT scan showed no renal alterations, with an adherent tumor near the parietal peritoneum suggestive of ectopic kidney. The puerperium was uneventful, and she was discharged from the hospital. CONCLUSION: In pregnancy, Müllerian malformations are a cause of serious obstetric complications, including uterine rupture. Timely diagnosis is decisive for the prevention of complications and prenatal management.

Embarazo gemelar espontáneo en útero bicorpóreo: una contribución a la experiencia / Spontaneous twin pregnancy in a bicorporeal uterus, a contribution to the experience

Resumen ANTECEDENTES: Las anomalías uterinas congénitas son consecuencia de la formación anormal, fusión o reabsorción de los conductos de Müller durante el desarrollo embriológico. El útero bicorne, unicorne y didelfo (U1, U2, U3 ESHRE-ESGE) son defectos en la unificación con una baja prevalencia en la población general y en la que tiene antecedente de aborto e infertilidad; se estima en alrededor de 0.4 a 1.1%, respectivamente. La presentación del caso puede aportar información útil relacionada con la evolución del embarazo y los desenlaces perinatales cuando aparece esta anomalía. CASO CLÍNICO: Paciente de 35 años, con amenorrea secundaria y prueba inmunológica de embarazo positiva, con antecedentes de dismenorrea primaria severa y anomalía uterina congénita compatible con útero bicorpóreo (U3 completo ESHRE-ESGE), bicorne completo (4a-AFS) y didelfo. El embarazo trascurrió sin complicaciones hasta la semana 37, cuando finalizó mediante cesárea, con hipotonía uterina que no ameritó transfusión de hemoderivados. CONCLUSIÓN: Los embarazos gemelares con útero bicorne son excepcionales y casi todos se logran espontáneamente; los que llegan a término finalizan mediante cesárea electiva.

Abstract BACKGROUND: Congenital uterine anomalies are the result of abnormal formation, fusion, or resorption of the Müllerian ducts during embryological development. The bicornuate, unicornuate and didelphic uterus (U1, U2, U3 ESHRE/ESGE), are unification defects with a low prevalence both in the general population and in the population with a history of abortion and infertility, is estimated at around 0.4 to 1.1%, respectively. The presentation of the case can provide useful information related to with the evolution of the pregnancy and the perinatal outcomes when this anomaly appears. CLINICAL CASE: A 35-year-old patient with secondary amenorrhea and a positive immunological pregnancy test with a history of severe primary dysmenorrhea and previous magnetic resonance imaging with a congenital uterine anomaly compatible with a bicorporeal uterus (U3 complete ESHRE-ESGE), complete bicornuate (4a /AFS) , uterus didelphys (ASRM). The pregnancy was uncomplicated until 37 weeks, when it was terminated by cesarean section, with uterine hypotonia that did not require transfusion of blood products. CONCLUSION: Twin pregnancy with bicornuate uterus is a rare entity, most of them have been achieved spontaneously and those that reach term have been resolved by elective cesarean section.

Correlation analysis of serum anti-Mullerian hormone level and ovarian interstitial blood flow in patients with polycystic ovary syndrome / 中国医师进修杂志

Objective:To investigate the correlation between the level of anti Mullerian hormone (AMH) in serum and ovarian interstitial blood flow in patients with polycystic ovary syndrome (PCOS).Methods:The clinical data of 88 patients with PCOS (PCOS group) admitted to Jiangshan Hospital of Traditional Chinese Medicine from June 2019 to June 2022 and 50 women of the same age who underwent physical examination during the same period (normal control group) were collected retrospectively. The patients in the PCOS group were divided into two subgroups according to the homeostatic model assessment for insulin resistance index (HOMA-IR) evaluated by the steady-state model. Among them, patients with HOMA-IR ≥ 2.69 were divided into the insulin resistance subgroup (PCOS-IR subgroup, 50 cases), and patients with HOMA-IR<2.69 were divided into the non-insulin resistance subgroup (PCOS-NIR subgroup, 38 cases). The level of AMH were measured by electrochemiluminescence, and ovarian interstitial blood flow indicators, including peak blood flow velocity (PSV), pulsation index (PI), and resistance index (RI) were monitored. Pearson correlation test was used to analyze the correlation between serum AMH and insulin resistance and ovarian interstitial blood flow. Meanwhile, Logistic linear regression model was used to analyze the influencing factors of ovarian interstitial blood flow in patients with PCOS.Results:The levels of AMH, HOMA-IR and PSV in the PCOS group were higher than those in the normal control group: (2.13 ± 0.84) μg/L vs. (0.84 ± 0.29) μg/L, 4.6(2.0, 8.4) vs. 1.8(0.5, 3.9), (10.05 ± 1.52) cm/s vs.(6.78 ± 0.89) cm/s; the PI and RI in the PCOS group were lower than those in the control group: (1.14 ± 0.26)% vs. (2.01 ± 0.53)%, (0.37 ± 0.18)% vs. (0.85 ± 0.33)%, there were statistical differences ( P<0.05). The levels of AMH, HOMA-IR and PSV in the PCOS-IR subgroup were higher than those in the PCOS-NIR subgroup: (2.68 ± 0.81) μg/L vs. (1.59 ± 0.43) μg/L, (10.74 ± 2.32) cm/s vs. (7.93 ± 1.90) cm/s, 7.2(3.1, 15.8) vs. 2.1(0.6, 5.3); the PI and RI in the PCOS-IR subgroup were lower than those in the PCOS-NIR subgroup: (0.88 ± 0.35)% vs. (1.52 ± 0.50)%, (0.29 ± 0.04)% vs. (0.51 ± 0.05)%, there were statistical differences ( P<0.05). Pearson correlation analysis showed that the level of AMH were positively correlated with PSV and HOMA-IR ( r = 0.694 and 0.540, P<0.05), but negatively correlated with PI and RI( r = - 0.687 and - 0.692, P<0.05). Logistic linear regression model analysis showed that AMH and HOMA-IR were risk factors for ovarian interstitial blood flow in patients with PCOS( P<0.05). Conclusions:The serum AMH level in patients with PCOS is abnormally high and increases with insulin resistance. At the same time, the imbalance of AMH expression plays an important role in the increase of abnormal ovarian interstitial blood flow.

Relationship between serum reproductive hormones and sperm parameters and surgical outcomes in Micro-TESE / 现代泌尿外科杂志

【Objective】 To investigate the relationship between serum reproductive hormones and sperm parameters and outcomes of micro-testicular sperm extraction (Micro-TESE). 【Methods】 Clinical data of 1 091 patients treated in our hospital during Jan. and Dec.2021 were retrospectively analyzed. According to the sperm concentration,the patients were divided into non-obstructive azoospermia (NOA) group (group A,n=418),normal sperm concentration group (group B,n=615),mild to moderate oligospermia group (group C,n=18),severe oligospermia group (group D,n=18),and obstructive azoospermia group (group E,n=22). In group A,244 cases treated with Micro-TESE were grouped into the sperm-acquired group (Micro-TESE positive group,n=82) and non-sperm-acquired group (Micro-TESE negative group,n=162),and according to the pathological types of testicular tissue,the patients were divided into normal testicular tissue with hypospermatogenesis group (HYPO group,n=129),maturation arrest group (MA group,n=10),and support-only cell syndrome group (SCO group,n=122). Differences in semen parameters and reproductive hormone levels were compared,and relationship between reproductive hormones and sperm parameters and Micro-TESE outcomes was determined with Pearson correlation analysis. 【Results】 In the sperm concentration subgroup,the testicular volume of group A was lower than that of group B and group E (P<0.05); the levels of follicle stimulating hormone (FSH) and luteinizing hormone (LH) in group A were the highest (P<0.05),but the level of testosterone (T) was the lowest (P<0.05); the levels of anti-mullerian hormone (AMH) and serum inhibin B (INHB) in group A were lower than those in group B and group E (P<0.05),the normal sperm morphology rate in group B was higher than that in group A and group E (P<0.05); the percentage of forward moving sperm in group B was the highest (P<0.05). Pearson correlation analysis revealed that sperm concentration,normal sperm morphology rate,and percentage of forward moving sperm were negatively correlated with age,FSH,LH (P<0.05),and positively correlated with testicular volume,T,AMH,and INHB (P<0.05). NOA patients were grouped according to testicular histology and pathology. The INHB in the SCO group was the smallest of the three groups (P<0.05); the FSH and LH levels in the SCO group were higher than those in the MA group (P<0.05),while the 17β-estradiol (E2) levels in the HYPO group were higher than those in the SCO group (P<0.05). NOA patients were grouped according to the results of Micro-TESE surgical treatment. There was a statistically significant difference in AMH and INHB levels between the Micro-TESE positive and negative groups (P<0.05). The binary logistic regression analysis of factors affecting the Micro-TESE outcomes of NOA patients showed AMH was negatively correlated with the Micro-TESE outcome (OR=0.904,95%CI:0.91-1.08,P<0.05). 【Conclusion】 Age,FSH,LH,AMH,and INHB are correlated with sperm concentration,normal sperm morphology rate,and percentage of forward moving sperm. The INHB level was the lowest in the SCO group. The results of Micro-TESE in patients with NOA can be predicted by serum AMH level.

Exploration of Mechanism of Xiaonang Tiaojing Decoction in Treating PCOS-IR Model Rats Based on AMH/AMHR ⅡSignaling Pathway / 中国实验方剂学杂志

ObjectiveTo investigate the mechanism of Xiaonang Tiaojing decoction（XNTJD）in improving polycystic ovary syndrome with insulin resistance（PCOS-IR）model rats based on anti-Müllerian hormone（AMH）/AMH type Ⅱ receptor（AMHRⅡ）signaling pathway. MethodForty-eight adult female SD rats were randomly divided into the blank group， model group， XNTJD group（11.4 g·kg-1·d-1） and Diane-35 group（0.21 g·kg-1·d-1）， PCOS-IR model was established by high-fat and high-sugar diet combined with letrozole in rats of all groups except the blank group， rats in the administration groups were given the corresponding dose of drugs by gavage for 15 days with an interval of 1 d every 4 d， normal saline of the same volume was given to the blank group and the model group. Estrous cycle was recorded daily during treatment. At the end of treatment， body weight and Lee's index were recorded， AMH， luteinizing hormone（LH）， LH/follicle stimulating hormone（FSH）， testosterone（T）and estradiol（E2）levels were measured by enzyme-linked immunosorbent assay（ELISA）， fasting plasma glucose（FPG）was measured by glucometer， fasting insulin（FINS） level was measured by radioimmunoassay（RIA）， and the insulin resistance index（HOMA-IR） and insulin sensitivity index（QUICKI）were calculated， triglyceride（TG）and total cholesterol（TC）levels were measured by automatic biochemical analyzer， hematoxylin-eosin（HE）staining was used to observe the morphological changes of the ovary， the levels of AMHRⅡ， bone morphogenetic protein-15（BMP-15）and Smad5 in ovarian tissues were detected by immunohistochemistry（IHC），Western blot was used to analyze the protein expression levels of AMHRⅡ， BMP-15 and Smad5. ResultCompared with the blank group， a large number of leukocytes were observed in the vaginal exfoliated cells of rats in the model group， mainly in diestrus， the levels of body weight， Lee's index， glucose-lipid metabolism indexes（FPG， FINS， HOMA-IR， TG， TC）， AMH and sex hormones（LH， LH/FSH， T）were significantly increased（P<0.01）， and QUICKI and E2 levels were significantly decreased（P<0.01）， there were more cystic bulges on the ovarian surface， more wet weight， more atretic and cystic dilated follicles in the ovarian tissues， and the thickness of granulosa cell layer was reduced without oocytes， the expression level of AMHRⅡ protein in ovarian tissues was significantly increased（P<0.01）， and the expression levels of BMP-15 and Smad5 proteins were significantly decreased（P<0.01）. Compared with the model group， the exfoliated cells in the vagina of rats treated with XNTJD group showed keratinocytes from the 5th to 6th day of treatment， and a stable estrous cycle gradually appeared， body weight， Lee's index， glucose-lipid metabolism indexes（FPG， FINS， HOMA-IR， TG， TC）， AMH and sex hormones（LH， LH/FSH， T）levels were significantly decreased（P<0.05， P<0.01）， QUICKI and E2 levels were significantly increased（P<0.01）， ovarian surface was smoother and lighter in wet weight， oocytes and mature follicles were observed in ovarian tissues， the thickness of granulosa cell layer increased and the morphology was intact， the expression levels of BMP-15 and Smad5 proteins were significantly increased（P<0.01）and the expression level of AMHRⅡ protein was significantly decreased（P<0.01）in ovarian tissues. ConclusionXNTJD may mediate the up-regulation of BMP-15 and Smad5 in ovarian tissues of PCOS-IR rats by down-regulating AMH/AMHRⅡ， thereby improving ovarian function， sex hormones and glucose-lipid metabolism levels in PCOS-IR rats.

Correlation analysis of vitamin D level and anti-Müllerian hormone in infertile female and the role in predicting pregnancy outcome / 北京大学学报(医学版)

OBJECTIVE@#To investigate the relationship between serum 25(OH)D and anti-Müllerian hormone (AMH) among infertile females and their predictive impacts on in vitro fertilization and embryo transfer pregnancy outcome.@*METHODS@#Totally 756 infertile females treated with assisted reproductive technology were enrolled and divided into three groups according to their vitamin D levels (group A with serum 25(OH)D≤10 μg/L, group B with serum (10-20) μg/L, and group C with serum ≥20 μg/L). The serum AMH levels were detected. The differences among the groups were analyzed, as well as the correlation between vitamin D levels and serum AMH levels in various infertility types (fallopian tube/male factor, polycystic ovary syndrome (PCOS), ovulation disorders excluded PCOS, endometriosis, unexplained infertility, and others). Also, the predictive roles of vitamin D and AMH in pregnancy outcome in all the infertile females were discussed.@*RESULTS@#(1) 87.7% of the enrolled females were insufficient or deficient in vitamin D. (2) The serum AMH levels in the three groups with different vitamin D levels were 1.960 (1.155, 3.655) μg/L, 2.455 (1.370, 4.403) μg/L, 2.360 (1.430, 4.780) μg/L and there was no significant difference in serum AMH levels among the three groups (P>0.05). (3) Serum 25(OH)D and AMH levels presented seasonal variations (P < 0.05). (4) There was no prominent correlation between the serum AMH level and serum 25(OH)D level in females of various infertility types after adjusting potential confounding factors [age, body mass index (BMI), antral follicle count (AFC), vitamin D blood collection season, etc.] by multiple linear regression analysis (P>0.05). (5) After adjusting for confounding factors, such as age, BMI, number of transplanted embryos and AFC, the results of binary Logistics regression model showed that in all the infertile females, the serum AMH level was an independent predictor of biochemical pregnancy outcome (P < 0.05) while the serum 25(OH)D level might not act as a prediction factor alone (P>0.05). In the meanwhile, the serum 25(OH)D level and serum AMH level were synergistic predictors of biochemical or clinical pregnancy outcome (P < 0.05).@*CONCLUSION@#Based on the current diagnostic criteria, most infertile females had vitamin D insufficiency or deficiency, but there was not significant correlation between serum 25(OH)D and ovarian reserve. While vitamin D could not be used as an independent predictor of pregnancy outcome in infertile females, the serum AMH level could predict biochemical pregnancy outcome independently or jointly with vitamin D.

Association of Dietary Protein on Level of Anti Mullerian Hormone (AMH) and Antral Follicle Count (AFC) in Patients of Infertility

Background : “Infertility is defined as the inability to conceive within a year with normal frequency of sexual intercourse and no contraceptives.” Relatively little is known about the effect of nutritional content on fertility. Objective : To study the correlation of level of AMH and AFC with dietary habits especially protein intake in patients of infertility. Material and Methods : It is hospital based study. Design of study : Cross sectional study. Place of study : Teerthankar Mahaveer Medical College & Research Centre, Moradabad, India . Number of Patients : 95 patients of infertility included in the study .Patients were between 30 and 45 years. Time Period : 18 months from January 2020-July 2021. Method : All cases underwent full history taking; clinical examination and all completed a questionnaire consisting of demographic characteristics, FFQ (Food Frequency Questionnaire). Main Outcome Measures : Moderate to high protein intake in diet corresponds to those having met their more than 20% calorie intake by protein had a higher mean AMH as well as had a higher mean AFC. Result : Based on the results of the current study the effects of higher protein intake was found to be significant on the level of ovarian reserve . Conclusion : This study suggests that good and healthy Nutrition, rich in proteins , in fertility treatment is required for better outcome and also helps in limiting the financial burden

Útero bicorporeo - doble cérvix con embarazo gemelar en una sola cavidad uterina: reporte de caso / Bicorporeo uterus - double cervix with twin pregnancy in a single uterine cavity: case report

El embarazo gemelar en una de las cavidades uterinas del útero bicorpóreo es una rara presentación clínica. La viabilidad de este tipo de embarazo es de baja probabilidad, dado que esta malformación puede condicionar parto prematuro, placentación anormal, restricción del crecimiento fetal o progresión anormal del trabajo de parto. Se presenta un caso de un útero bicorpóreo con doble cérvix y una gestación gemelar en unas de las cavidades uterinas, con muerte fetal de uno de los fetos y parto pretérmino de 28 semanas del otro gemelo. Dada la rareza de la presentación se describen el caso y los hallazgos imagenológicos representativos.

Twin pregnancy in one of the uterine cavities of the bicoporous uterus is a rare clinical presentation. The viability of this type of pregnancy is unlikely since this malformation can lead to premature delivery, abnormal placentation, fetal growth restriction or abnormal progression of labor. We present a case of bicoporous uterus with double cervix with twin pregnancy in one of the uterine cavities, with stillbirth of one of the fetuses and preterm delivery of the other twin at 28 weeks. Given the rarity of the presentation, the case and representative imaging findings are described.

Microsurgical technique for reconstruction of Hemi-uterus with non-communicating rudimentary uterine horns / Técnica microquirúrgica para reconstrucción de Hemi-útero con cuernos uterinos rudimentarios no comunicantes

Abstract Case description: Three cases of patients diagnosed with Hemi-uterus with non-communicating functional rudimentary cavity are reported. Clinical findings: Adolescent patients with the presence of disabling pelvic pain during menstrual periods, in addition to depressive states and functional disability secondary to pain. Treatment and outcome: Reconstruction of Hemi-uterus with non-communicating functional rudimentary cavity using a microsurgical technique, without the need for non-communicating horn resection. The patients were assessed at the 1st, third, and sixth months, showing a marked improvement in their dysmenorrhea. In addition, there was an improvement in the depressive state; there was no change in the volume of her menstrual bleeding, and one of these patients achieved pregnancy with normal prenatal control and cesarean delivery, obtaining a full-term newborn without complications. Clinical Relevance: Description of a novel microsurgical technique for the management of the hemi-uterus with a non-communicating functional rudimentary cavity, which could benefit not only the reduction of associated symptoms but could also become an alternative to improve fertility in these patients.

Resumen Descripción del caso: Se reportan tres casos de pacientes con diagnóstico de Hemiútero con cavidad rudimentaria funcional no comunicante. Hallazgos clínicos: Pacientes adolescentes con presencia de dolor pélvico incapacitante durante los períodos menstruales, además de estados depresivos e incapacidad funcional secundaria al dolor. Tratamiento y resultado: Reconstrucción de Hemiútero con cavidad rudimentaria funcional no comunicante mediante técnica microquirúrgica, sin necesidad de resección de cuerno no comunicante. Las pacientes fueron evaluadas al 1, 3 y 6 mes, mostrando una marcada mejoría en su dismenorrea. Además, hubo mejoría en el estado depresivo; no hubo cambio en el volumen de su sangrado menstrual, y una de estas pacientes logró el embarazo con control prenatal normal y parto por cesárea, obteniendo un recién nacido a término sin complicaciones. Relevancia clínica: Descripción de una novedosa técnica microquirúrgica para el manejo del Hemiútero con cavidad rudimentaria funcional no comunicante, que podría beneficiar no solo la reducción de los síntomas asociados, sino que podría convertirse en una alternativa para mejorar la fertilidad en estas pacientes

Accessory and cavitated uterine mass: A rare cause of dysmenorrhea and chronic pelvic pain

Accessory and cavitated uterine mass (ACUM) is a rare developmental uterine anomaly that is said to develop due to duplication of ductal Mullerian tissue. It is an accessory cavity lying along the lateral wall of the uterus near the site of attachment of the round ligament and presents in young females with pelvic pain and dysmenorrhea. Histologically, the cavity is lined with endometrium and smooth muscle bundles similar to the myometrium. Ultrasound is helpful in the identification of the lesion. MRI of the pelvis is the imaging modality of choice for diagnosis. Laparoscopy and histopathology remain confirmatory. This report describes a case of ACUM in a young female who was managed by surgical excision of the mass.

Mayer-Rokitansky-Kuster-Hauser syndrome – An unusual case of primary amenorrhea

The onset of menstruation is a significant milestone of sexual maturation in a girl child. Although there are numerous causes of primary amenorrhea, Mayer-Rokitansky-Küster-Hauser syndrome, also known as Müllerian aplasia, is one of the extremely rare causes of primary amenorrhea which is usually picked up by suitable imaging techniques like magnetic resonance imaging. Affected females have a normal female hormonal function and a normal karyotype but non-functional vagina and uterus which make it an interesting entity. Although specific treatment is not known for this entity except for vaginoplasty and creation of a neovagina for sexual gratification, the clinical and diagnostic workup of these patients has evolved in recent years and infertility treatment and child-bearing through in vitrofertilization have become a part of the long-term treatment plan for these females.

Embarazo ectópico en cuerno rudimentario izquierdo de útero unicorne con embarazo de 15 semanas: reporte de un caso / Ectopic pregnancy in rudimentary left horn of unicorne uterus with pregnancy of 15 weeks: a case report

INTRODUCCIÓN: El embarazo ectópico en el cuerno rudimentario de un útero unicorne tiene una incidencia de 1 en 76.000 embarazos. La aproximación diagnóstica se realiza con la ecografía y como estudio complementario con la resonancia magnética. El diagnóstico temprano con tratamiento oportuno es fundamental para la prevención de la morbimortalidad materna asociada. El objetivo es describir el diagnóstico y el tratamiento temprano de un caso de embarazo ectópico de 15 semanas en cuerno rudimentario no comunicante de útero unicorne. CASO CLÍNICO: Mujer de 38 años con embarazo de 15 semanas, asintomática, que ingresa al servicio de urgencias referida desde el servicio de ecografía por sospecha de embarazo ectópico. Se realizan ecografía y resonancia magnética que muestran embarazo con feto único de 15 semanas en cuerno uterino izquierdo rodeado de miometrio, sin comunicación con la cavidad endometrial. Con impresión diagnóstica de embarazo ectópico cornual en paciente con malformación mülleriana, se realizó manejo quirúrgico que confirmó útero unicorne con embarazo ectópico en cuerno rudimentario no comunicante. CONCLUSIONES: El embarazo ectópico en un cuerno rudimentario de útero unicorne es infrecuente y presenta un alto riesgo de rotura, con aumento de la morbimortalidad obstétrica. El tratamiento estándar, al igual que la confirmación diagnóstica, es la escisión quirúrgica completa.

INTRODUCTION: Ectopic pregnancy in the rudimentary horn of a unicornuate uterus has an incidence of 1 in 76,000 pregnancies; the diagnostic approach is carried out with ultrasound and magnetic resonance imaging as a complementary study; Early diagnosis with timely treatment is essential for the prevention of associated maternal morbidity and mortality. The objective is to describe the early diagnosis and treatment of a case of 15-week ectopic pregnancy in a rudimentary non-communicating horn of the unicornuate uterus. CASE REPORT: A 38-year-old patient with an asymptomatic 15-week pregnancy was admitted to the emergency department, referred to the ultrasound service for suspected ectopic pregnancy. Ultrasound and magnetic resonance imaging were performed with pregnancy with a single fetus of 15 weeks in the left uterine horn surrounded by myometrium, without communication with the endometrial cavity. With a diagnostic impression of cornual ectopic pregnancy in a patient with a Müllerian malformation, a surgical management was performed where a unicornuate uterus with a rudimentary non-communicating ectopic horn was confirmed. CONCLUSIONS: Ectopic pregnancy in rudimentary horn of the unicornuate uterus is rare, it presents a high risk of rupture with increased obstetric morbidity and mortality. The standard treatment as well as the diagnostic confirmation is complete surgical excision.

Pregnancies in anomalous uterus: series of three cases

Uterine malformations can result from various alterations in the normal development of Mullerian ducts that make up a diverse category of congenital anomalies. In most cases, the defect remains undiagnosed and exhibits the normal number of chromosomes. Very few malformations require any intervention after thorough investigations. The most frequently used classification by the American Society for Reproductive Medicine is comprehensive, but does not include cervical or vaginal malformations. The European Society of Human Reproduction and Embryology has postulated a consensus that precisely shows independent cervical malformations.

Tumor mülleriano mixto maligno / Malignant mixed müllerian tumor

RESUMEN Introducción: El tumor mülleriano mixto maligno es una neoplasia uterina infrecuente, agresiva y se caracteriza por su histología por contener elementos malignos sarcomatosos y carcinomatosos. Objetivo: Describir un caso de tumor mülleriano mixto maligno y revisar la literatura sobre el tema. Caso clínico: Paciente femenina de 63 años de edad, color de la piel negra, obesa e hipertensa con tratamiento, antecedentes de mastectomía derecha seis meses atrás, acudió a consulta por presentar aumento de volumen abdominal, dolor en bajo vientre y sangramiento uterino. Con la administración de anestesia combinada (general orotraqueal y regional epidural continua) se resecó un fibroma uterino gigante y se confirmó el diagnóstico de un tumor mülleriano mixto maligno. Conclusiones: El tumor mülleriano mixto maligno se debe sospechar en mujeres de edad avanzada con sangrado genital acompañado de masa pélvica. El estudio anatomopatológico es de elección para el diagnóstico definitivo de esta neoplasia rara y muy agresiva. La cirugía, la radioterapia y la quimioterapia son las opciones terapéuticas más aceptadas, sin embargo, en mujeres con tumor en estadios avanzados, independiente del tratamiento proporcionado, la tasa de supervivencia es corta.

ABSTRACT Introduction: The malignant mixed müllerian tumor is an infrequent, aggressive uterine neoplasm and is histologically characterized by containing sarcomatous and carcinomatous malignant elements. Objective: To describe a case of a malignant mixed Müllerian tumor and to review the literature on the subject. Case report: A 63-year-old black female patient, obese and hypertensive under treatment, with a history of right mastectomy six months ago, who was consulted due to increased abdominal volume, lower abdominal pain and uterine bleeding. With the administration of combined anesthesia (general orotracheal and continuous epidural regional) a giant uterine fibroid was resected and the diagnosis of a malignant mixed Müllerian tumor was confirmed. Conclusions: Malignant mixed Müllerian tumor should be suspected in elderly women with genital bleeding accompanied by a pelvic mass. The pathological study is of choice for the definitive diagnosis of this rare and potentially aggressive neoplasm. Surgery, radiotherapy and chemotherapy are the most accepted therapeutic options, however, in women with advanced stage tumors, regardless of the treatment provided, the survival rate is short.