scholarly journals Amenorrea primaria

2009 ◽  
Vol 60 (1) ◽  
pp. 57-67
Author(s):  
Janer Sepúlveda-Agudelo ◽  
Miguel Ángel Alarcón-Nivia ◽  
Hermes Jaimes-Carvajal
Keyword(s):  
Turner Syndrome ◽  
Hypogonadotropic Hypogonadism ◽  
Kallmann Syndrome ◽  
Primary Amenorrhea ◽  
Menstrual Disorders ◽  
Palabras Clave

Objetivo: se hace una revisión detallada de la amenorreaprimaria, teniendo como base la clasificación propuesta por Mashchak CA y col. de acuerdo con la presencia o ausencia del desarrollo mamario y la presencia o no de útero, por ser la de mayor utilidad para el enfoque de manejo de las pacientes con amenorrea primaria.Metodología: se realizó una búsqueda de la literatura publicada en inglés a través de MEDLINE y OVID, usando como palabras clave: amenorrhea, primary amenorrhea, menstrual disorders, Turner syndrome, Kallmann syndrome, Prader-Willi síndrome, hypogonadotropic-hypogonadism; y se clasificó la información como soporte de la presente revisión, realizando resúmenes para su análisis.Resultados: la amenorrea primaria puede ser causada por una variedad de alteraciones que incluyen anormalidades müllerianas, gonadales, hipofisiarias, hipotalámicas, adrenales y tiroideas, o disfunciones hormonales en estos diferentes niveles. Estas anormalidades pueden ser congénitas por defectos cromosómicos o genéticos, o adquiridas, por lo tanto, es importante realizar un diagnóstico certero de esta patología para llevar a cabo un enfoque terapéutico adecuado, con el fin de disminuir todas las consecuencias que la enfermedad puede causar. Conclusiones: el tratamiento de las pacientes con amenorrea primaria debe ser individualizado, de acuerdo con las posibilidades terapéuticas de cada paciente, pero existen unas preguntas generales que tienen todas las pacientes o sus familiares y son relacionadas con la menstruación y los ciclos menstruales espontáneos posteriores, fertilidad, sexualidad y posibilidad de coitos con penetración vaginal satisfactoria.

Turner syndrome and pituitary adenomas: a case report and review of literature

2017 ◽  
Vol 30 (2) ◽  
Author(s):  
Tiffany Yeh ◽  
Angela Ganan Soto ◽  
Jose Bernardo Quintos ◽  
Lisa Swartz Topor
Keyword(s):  
Turner Syndrome ◽  
Pituitary Adenomas ◽  
Case Reports ◽  
Hypogonadotropic Hypogonadism ◽  
Brain Mri ◽  
Laboratory Evaluation ◽  
Primary Amenorrhea ◽  
Pituitary Hormone ◽  
Pituitary Macroadenoma ◽  
Hypergonadotropic Hypogonadism

AbstractBackground:Turner syndrome (TS) is the most common sex chromosome abnormality in females, typically associated with primary amenorrhea and premature ovarian failure due to gonadal dysgenesis. The association of TS with hypopituitarism is an uncommon finding. The objective of the study was to describe an adolescent with TS with hypergonadotropic hypogonadism and subsequent hypogonadotropic hypogonadism.Case presentation:A 16-year-old female with primary amenorrhea was diagnosed with TS based on karyotype 45,XO. Other laboratory values included FSH 45.52 IU/L, LH 17.4 IU/L, undetectable estradiol, and prolactin 1.08 nmol/L. Two months later and before treatment, she presented with severe headache and a new left cranial nerve VI palsy. Brain MRI showed a 2.7-cm hemorrhagic pituitary macroadenoma expanding the sella. Laboratory evaluation showed FSH 5.9 IU/L, LH 0.9 IU/L, prolactin 0.09 nmol/L, and GH 1.03 ng/mL. She underwent transphenoidal hypophysectomy, and pathology revealed pituitary adenoma with immunohistochemical staining positive for growth hormone and prolactin. She subsequently developed multiple pituitary hormone deficiencies. Review of the literature identified eight case reports of women with TS who developed pituitary adenomas.Conclusions:This case illustrates an uncommon co-occurrence of TS and pituitary macroadenoma. Sequential gonadotropin measurements demonstrate the evolution of hypergonadotropic hypogonadism into hypogonadotropic hypogonadism due to hemorrhagic pituitary macroadenoma.

scholarly journals Hormonal regulation of menstrual function in patients of reproductive age with acromegaly

2019 ◽  
Vol 15 (4) ◽  
pp. 59-64 ◽  
Author(s):  
Svetlana Yu. Vorotnikova ◽  
Larisa K. Dzeranova ◽  
Irina V. Stanoevich ◽  
Ekaterina A. Pigarova ◽  
Elena N. Andreeva ◽  
...  
Keyword(s):  
Hormonal Regulation ◽  
Hypogonadotropic Hypogonadism ◽  
Mass Effect ◽  
Reproductive Age ◽  
Menstrual Disorders ◽  
Serum Samples ◽  
Menstrual Function ◽  
Healthy Women ◽  
Excessive Secretion ◽  
Preliminary Extraction

Background: The frequency of menstrual disorders in patients with acromegaly is 4084% and are caused by three main reasons the development of normal or hypogonadotropic hypogonadism due to hyperprolactinemia or a mass effect of the tumor and direct effects of GH and IGF-1 on the reproductive system. Nevertheless the exact mechanisms of reproductive dysfunction are not clear now. Hypothalamic structures play significant role in the regulation of hypothalamic-pituitary-ovary axis, so its important to study key neuropeptides and evaluate their effects to the pathogenesis of ovarian dysfunction during excessive secretion of growth hormone. Aim: The aim of the work is to study the hormonal regulation of menstrual function in patients of reproductive age with acromegaly in the active stage of the disease. Material and methods: The study included patients with a confirmed diagnosis of acromegaly and healthy women, comparable in age and BMI. Blood serum samples were taken in the morning (89 hours) on an empty stomach for 35 days of the menstrual cycle or on any day with amenorrhea and frozen at -70C. The hormonal study was carried out by an enzyme immunoassay, in the case of a kisspeptin, with the preliminary extraction of serum samples. Results: The study included 31 patients with acromegaly and 15 healthy women. Between groups there was a statistically significant decrease in levels of LH (p = 0.001), FSH (p = 0.09), inhibin B (p = 0.003), and kisspeptin (p = 0.00005). The frequency of hyperprolactinemia in the cohort of patients was 51.6%. During the correlation analysis, a negative dependence of kisspeptin on the levels of GH and IGF-1 was detected (r = -0.54, p = 0.002 and r = -0.63, p = 0.0002). Conclusions: The severity of the central depression of regulation of menstrual function in patients with acromegaly may be due to the degree of disease activity.

A Rare Case of Coexistence of Pituitary Stalk Interruption Syndrome and Mayer-Rokitansky-Küster-Hauser Syndrome

2020 ◽  
Author(s):  
Wanlu Ma ◽  
Xi Wang ◽  
jiangfeng mao ◽  
Min Nie ◽  
Xueyan Wu
Keyword(s):  
Computed Tomography ◽  
Differential Diagnosis ◽  
Rare Case ◽  
Hypogonadotropic Hypogonadism ◽  
Pituitary Stalk ◽  
Primary Amenorrhea ◽  
Case Presentation ◽  
Mrkh Syndrome ◽  
Breech Delivery ◽  
Prepubertal Girls

Abstract Background Pituitary stalk interruption syndrome (PSIS) is a rare congenital pituitary anatomical disorder. Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is characterized by congenital absence of the uterus, cervix, and part of the vagina in phenotypically normal 46, XX females. Case presentation A young woman was initially diagnosed as MRKH syndrome based on primary amenorrhea, 46, XX karyotype, and absence of uterus or vagina. Further investigation revealed breech delivery, short stature, hypogonadotropic hypogonadism, interrupted pituitary stalk on pituitary MRI, which led to the diagnosis of PSIS. After a 12-month treatment with estradiol, no signs of uterus or vagina were found on pelvic computed tomography.Conclusions We highlight the importance of considering PSIS in the differential diagnosis of suspected MRKH syndrome in prepubertal girls or girls with delayed or absent puberty, when no uterus is visualized on imaging.

Inhibin B in adolescents and young adults with Turner syndrome

2015 ◽  
Vol 28 (11-12) ◽  
Author(s):  
Maria Francesca Messina ◽  
Tommaso Aversa ◽  
Giuseppina Salzano ◽  
Daria Costanzo ◽  
Concetta Sferlazzas ◽  
...  
Keyword(s):  
Turner Syndrome ◽  
Ovarian Reserve ◽  
Ovarian Function ◽  
Ovarian Tissue ◽  
Pubertal Development ◽  
Inhibin B ◽  
Control Group ◽  
Primary Amenorrhea ◽  
Menstrual Cycles ◽  
Reliable Marker

AbstractPrimary gonadal failure may occur in most individuals with Turner syndrome (TS). Since ovaries in TS girls undergo premature apoptosis and cryopreservation of ovarian tissue is now feasible, it would be useful to identify a reliable marker of ovarian reserve in these patients. We planned to evaluate ovarian function in a group of TS patients by measuring both traditional markers and inhibin B and to compare these results with those of a control group.We enrolled 23 patients with TS and 17 age-matched healthy girls. The median age of our TS patients was 17.6 years. Three out of the 23 patients (13%) showed spontaneous pubertal development and regular menstrual cycles; the remaining 20 (86.9%) presented with primary amenorrhea.The median level of inhibin B in the TS patients with primary amenorrhea was 42 pg/mL and did not differ significantly among the different subgroups in relation to karyotype. The median inhibin B level in the control group was significantly higher than in the TS girls with primary amenorrhea (83 vs. 42 pg/mL, p<0.00001). In the three patients with TS and spontaneous menstrual cycles, the inhibin B levels were significantly higher when compared to the values of the TS girls with primary amenorrhea.TS patients with primary amenorrhea have significantly lower levels of inhibin B than TS girls with spontaneous puberty and healthy controls. Inhibin B does not correlate with follicle-stimulating hormone/luteinizing hormone. If our results are confirmed in further studies, inhibin B could become a first-line screening test for assessing ovarian reserve and a longitudinal marker of the possible decline of ovarian function in TS.

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