Insuficiencia ovárica primaria en adolescentes: revisión de la literatura a propósito de un caso clínico / Primary ovarian insufficiency in adolescents: review of the literature regarding a clinical case

El objetivo de este manuscrito es realizar una revisión y actualización de la literatura de la insuficiencia ovárica primaria (IOP) en población adolescente, a partir del diagnóstico, manejo y seguimiento de un caso clínico. La insuficiencia ovárica primaria se define como la menopausia en una mujer antes de los 40 años, acompañada de amenorrea, hipogonadismo hipergonadotrópico e infertilidad. Su prevalencia varía entre 1 a 2%, y en mujeres menores de 20 años su prevalencia es un caso de cada 10,000. Aunque se sabe que muchas afecciones pueden llevar a una IOP, la más común es la causa idiopática. La presentación clínica es diversa, y varios trastornos diferentes pueden también, llevar a esta condición. CASO CLÍNICO: Se presenta el caso de una adolescente de 17 años, previamente sana, con historia de amenorrea secundaria, no embarazada, con examen físico general y ginecológico normal. Se solicita estudio analítico complementario resultando con niveles de hormona folículo estimulante (FHS), estradiol (E2) y hormona antimülleriana (AMH) compatibles con una insuficiencia ovárica como la observada en la posmenopausia. Se inicia terapia hormonal (TH) clásica con estradiol y progesterona, siendo posteriormente reemplazada por anticoncepción hormonal combinada (AHC) oral, coincidente con el inicio de vida sexual, con respuesta favorable y sangrados regulares. La IOP tiene graves consecuencias para la salud incluyendo trastornos psicológicos como angustia, síntomas depresivos o depresión, infertilidad, osteoporosis, trastornos autoinmunes, cardiopatía isquémica, y un mayor riesgo de mortalidad. La enfermedad de Hashimoto es el trastorno autoinmune más frecuente asociado a la IOP. Su tratamiento y diagnóstico deben establecerse de forma precoz para evitar consecuencias a largo plazo. La terapia con estrógenos es la base del tratamiento para eliminar los síntomas de la deficiencia de estrógenos, además de evitar las consecuencias futuras del hipogonadismo no tratado. También el manejo debe incluir los siguientes dominios: fertilidad y anticoncepción, salud ósea, problemas cardiovasculares, función psicosexual, psicológica y neurológica, informando a los familiares y a la paciente sobre la dimensión real de la IOP y la necesidad de tratamiento multidisciplinario en muchos casos. CONCLUSIÓN: El caso presentado, pese a ser infrecuente, permite abordar de manera sistematizada el diagnostico de IOP y evaluar alternativas de manejo plausibles para evitar graves consecuencias en la salud, así como conocer respuesta clínica y de satisfacción de la adolescente.

The objective of this manuscript is to review and update the literature on primary ovarian insufficiency (POI) in an adolescent population, based on the diagnosis, management and follow-up of a clinical case. Primary ovarian insufficiency is defined as menopause in a woman before the age of 40, accompanied by amenorrhea, hypergonadotropic hypogonadism, and infertility. Its prevalence varies between 1 to 2%, and in women under 20 years of age its prevalence is one case in every 10,000. Although it is known that many conditions can lead to POI, the most common is the idiopathic cause. The clinical presentation is diverse, and several different disorders can also lead to this condition. CLINICAL CASE: The case of a 17-year-old adolescent, previously healthy, with a history of secondary amenorrhea, not pregnant, with a normal general physical and gynecological examination is presented. A complementary analytical study is requested, resulting in levels of follicle stimulating hormone (FHS), estradiol (E2) and anti-müllerian hormone (AMH) compatible with ovarian insufficiency such as that observed in postmenopause. Classic hormonal therapy (HT) with estradiol and progesterone was started, later being replaced by combined hormonal contraception (CHC), coinciding with the beginning of sexual life, with a favorable response and regular bleeding. POI has serious health consequences including psychological disorders such as distress, depressive symptoms or depression, infertility, osteoporosis, autoimmune disorders, ischemic heart disease, and an increased risk of mortality. Hashimoto's disease is the most common autoimmune disorder associated with POI. Its treatment and diagnosis must be established early to avoid long-term consequences. Estrogen therapy is the mainstay of treatment to eliminate the symptoms of estrogen deficiency, in addition to avoiding the future consequences of untreated hypogonadism. Management should also include the following domains: fertility and contraception, bone health, cardiovascular problems, psychosexual, psychological and neurological function, informing family members and the patient about the real dimension of POI and the need for multidisciplinary treatment in many cases. CONCLUSION: The case, although infrequent, allows a systematic approach to the diagnosis of POI and evaluate plausible management alternatives to avoid serious health consequences, as well as to know the clinical response and satisfaction of the adolescent.

Quiste de bolsa de Rathke e hiperprolactinemia, una causa infrecuente de amenorrea primaria / Rathke cleft cysts and hyperprolactinemia, an uncommon cause of primary amenorrhea

El quiste de la bolsa de Rathke es una lesión epitelial benigna de la región selar, formada a partir de remanentes embrionarios. La mayoría de los casos son asintomáticos, aunque pudiera presentarse con cefalea, disfunción hipofisaria y trastornos visuales, muy infrecuentemente como apoplejía hipofisaria. Se presenta el caso de una paciente que, habiendo presentado amenorrea primaria, se le realiza el diagnóstico de quiste de la bolsa de Rathke con hiperprolactinemia, logrando menarquia luego del tratamiento con cabergolina.

Rathke's cyst is a benign epithelial lesion of the sellar region, formed from embryonic remnants. Most cases are asymptomatic although it could present with headache, pituitary dysfunction and visual disorders, very infrequently as pituitary stroke. We present the case of a patient who, having presented primary amenorrhea, is diagnosed with Rathke's cyst with hyperprolactinemia, achieving menarche after treatment with cabergoline.

Disgenesia gonadal pura 46 XX, una causa infrecuente de hipogonadismo: presentación de un caso / Pure gonadal dysgenesis 46 XX, an ucommon cause of hypogonadism: case presentation

El término disgenesia gonadal pura hace referencia a mujeres fenotípicas con infantilismo sexual, amenorrea primaria, hábito eunucoide y un cariotipo 46, XX o 46, XY sin anomalías cromosómicas. Puede asociarse a complicaciones como osteoporosis y síndrome metabólico, elevando el riesgo cardiovascular. Se presenta una paciente femenina de 16 años y 8 meses de edad que acude a consulta de endocrinología por presentar amenorrea primaria.

The term pure gonadal dysgenesis refers to phenotypic women with sexual infantilism, primary amenorrhea an d the eunucoid habit and a 46, XX or 46, XY karyotype without chromosomal abnormalities. It can be associated with complications such as osteoporosis and metabolic syndrome, increasing cardiovascular risk. We present a female patient of 16 years and 8 months of age who attended endocrinology clinic for presenting primary amenorrea.

Primary amenorrhea associated with hyperprolactinemia in polyglandular autoimmune syndrome type II: A case report / Amenorreia primária associada com hiperprolactinemia em síndrome poliglandular autoimune tipo 2: relato de caso

Abstract Polyglandular autoimmune syndrome type II (PGA-II) is a rare immunoendocrinopathy syndrome characterized by the occurrence of autoimmune Addison disease along with diabetes mellitus type 1 and/or autoimmune thyroid disease. Here, we report the case of a 23-year-old female with PGA-II who was followed up at the dermatology and endocrinology clinics of the Universidade Federal do Triângulo Mineiro, located in the state of Minas Gerais, Brazil. First, the patient presented diffuse skin hyperpigmentation, vitiligo; and in sequence, due to vomiting, appetite and weight loss, hypoglycemia, amenorrhea, and galactorrhea, the patient was then diagnosed with PGA-II. The patient also presented intense hyperprolactinemia due to primary hypothyroidism. The late diagnosis of PGA-II is frequent because the disorder is uncommon and has non-specific clinical manifestations. This report emphasizes the significance of a timely diagnosis and appropriate treatment to reduce morbidity and mortality associated with these diseases, especially Addison disease. The present study reports a rare case of a patient with PGA-II with primary amenorrhea associated with hyperprolactinemia.

Resumo A síndrome poliglandular autoimune tipo 2 (SPGA-2) é uma síndrome de imunoendocrinopatia rara caracterizada por doença de Addison autoimune associada à diabetes mellitus tipo 1 e/ou doenças tireoidianas autoimunes. Relatamos aqui o caso de uma paciente de 23 anos de idade com SPGA-2 que foi acompanhada nos ambulatórios de dermatologia e endocrinologia da Universidade Federal do Triângulo Mineiro, localizada no estado de Minas Gerais, Brasil. Primeiramente, a paciente apresentou hiperpigmentação cutânea difusa e vitiligo; posteriormente, por apresentar vômitos, hiporexia, perda ponderal, hipoglicemia, amenorreia e galactorreia, foi diagnosticada com SPGA-2. A paciente apresentou também intensa hiperprolactinemia secundária apenas ao hipotireoidismo primário. É comum o diagnóstico tardio da SPGA-2, pois a doença é rara e apresenta manifestações clínicas inespecíficas. Este relato de caso enfatiza a importância do diagnóstico e tratamento precoces como objetivo de reduzir a morbimortalidade associada a essas doenças, especialmente à doença de Addison. O presente estudo descreve um caso raro de uma paciente com SPGA-2 com amenorreia primária associada a hiperprolactinemia.

Menstruation disorders in adolescents with eating disorders ? target body mass index percentiles for their resolution / Distúrbios menstruais em adolescentes com transtornos alimentares ? meta de percentil de índice de massa corporal para resolução dos distúrbios menstruais

Objective To analyse the progression of body mass index in eating disorders and to determine the percentile for establishment and resolution of the disease. Methods A retrospective descriptive cross-sectional study. Review of clinical files of adolescents with eating disorders. Results Of the 62 female adolescents studied with eating disorders, 51 presented with eating disorder not otherwise specified, 10 anorexia nervosa, and 1 bulimia nervosa. Twenty-one of these adolescents had menstrual disorders; in that, 14 secondary amenorrhea and 7 menstrual irregularities (6 eating disorder not otherwise specified, and 1 bulimia nervosa). In average, in anorectic adolescents, the initial body mass index was in 75th percentile; secondary amenorrhea was established 1 month after onset of the disease; minimum weight was 76.6% of ideal body mass index (at 4th percentile) at 10.2 months of disease; and resolution of amenorrhea occurred at 24 months, with average weight recovery of 93.4% of the ideal. In eating disorder not otherwise specified with menstrual disorder (n=10), the mean initial body mass index was at 85th percentile; minimal weight was in average 97.7% of the ideal value (minimum body mass index was in 52nd percentile) at 14.9 months of disease; body mass index stabilization occured at 1.6 year of disease; and mean body mass index was in 73rd percentile. Considering eating disorder not otherwise specified with secondary amenorrhea (n=4); secondary amenorrhea occurred at 4 months, with resolution at 12 months of disease (mean 65th percentile body mass index). Conclusion One-third of the eating disorder group had menstrual disorder − two-thirds presented with amenorrhea. This study indicated that for the resolution of their menstrual disturbance the body mass index percentiles to be achieved by female adolescents with eating disorders was 25-50 in anorexia nervosa, and 50-75, in eating disorder not otherwise specified. .

Objetivo Analisar a progressão do índice de massa corporal nos transtornos alimentares e determinar o percentil para estabelecimento e resolução da doença. Métodos Estudo retrospectivo descritivo, com análise dos processos de adolescentes com transtorno alimentar. Resultados Das 62 adolescentes com distúrbio alimentar, 51 apresentavam distúrbio sem outra especificação, 10 anorexia nervosa, e uma bulimia. Vinte e uma adolescentes apresentavam distúrbios menstruais, sendo 14 amenorreia secundária, 7 irregularidades menstruais (6 distúrbio alimentar sem outra especificação e 1 bulimia). Em média, nas anoréticas: índice de massa corporal inicial - percentil 75, instalação da amenorreia secundária com um mês de doença, peso mínimo 76,6% do índice de massa corporal ideal, no percentil 4), com 10,2 meses de doença, resolução da amenorreia aos 24 meses com recuperação ponderal média de 93,4% do peso ideal. No distúrbio alimentar sem outra especificação com distúrbio menstrual (n=10), o índice de massa corporal inicial médio no percentil 85, peso mínimo, em média, 97,7% do valor de peso ideal (média no percentil 52 de índice de massa corporal), aos 14,9 meses, estabilização ponderal aos 1,6 anos, percentil médio de índice de massa corporal de 73. Na perturbação do comportamento alimentar sem outra especificação com amenorreia secundária (n=4): amenorreia secundária aos 4 meses, resolução aos 12 meses (média no percentil 65 do índice de massa corporal). Conclusão Um terço do grupo com transtornos alimentares teve distúrbios menstruais − sendo dois terços com amenorreia secundária. Este estudo indicou que, para resolução ...

Avaliação da densidade mineral óssea em adolescentes do sexo feminino com transtorno alimentar / Evaluation of bone mineral density in female adolescents with eating disorders

OBJETIVO: Avaliar a densidade mineral óssea (DMO) lombar em meninas com transtorno alimentar no diagnóstico e após seis meses e um ano de tratamento. SUJEITOS E MÉTODOS: Estudo prospectivo com 35 adolescentes do sexo feminino, portadoras de AN ou TANE acompanhadas por um ano. A densitometria óssea (DO) da coluna lombar L1-L4 pelo método de absorciometria com raios X de dupla energia (DXA) foi realizada no início, após seis meses e um ano de tratamento. RESULTADOS: Houve aumento do peso, da altura e do índice de massa corporal (IMC), progressão da idade óssea (p < 0,001), e 70% das adolescentes com amenorreia secundária restabeleceram os ciclos menstruais. No entanto, não houve diferença significativa do escore Z da DO lombar ao longo de um ano (p = 0,76). CONCLUSÃO: A recuperação da DMO não ocorre ao mesmo tempo em que a restauração do eixo hipotalâmico-hipofisário-gonadal.

OBJECTIVE: To evaluate lumbar bone mineral density (BMD) in girls with eating disorders in the beginning of the treatment, at six months, and after one year of treatment. SUBJECTS AND METHODS: This prospective study involved 35 female adolescents with AN or EDNOS treated during one year. Lumbar (L1-L4) bone mineral density by DXA was performed in the beginning of treatment, at six months, and after one year of treatment. RESULTS: There was improvement in weight, length, BMI, bone age (p < 0.001), and 70% of the adolescents with secondary amenorrhea had their menstrual cycles restored. However, the Z-score of lumbar BMD did not show differences during one year of follow-up (p = 0.76). CONCLUSION: The recovery of BMD does not occur together with the restoration of hypothalamic-pituitary-gonadal axis.

Ovarian hemangioma with stromal luteinization and HCG-producing mononucleate and multinucleate cells of uncertain histogenesis: A rare co-existence with therapeutic dilemma.

A 21 year old female presented with amenorrhea, hirsutism and change in voice along with an elevated serum β-HCG (human chorionic gonadotrophin) level and normal CA-125 level. Laparotomy revealed an enlarged right ovary measuring 6 × 5 × 1 cms with presence of an ovarian hemangioma along with stromal luteinization and HCG producing mononucleate as well as multinucleate cells of uncertain histogenesis on histopathological examination. Immunohistochemistry for inhibin and calretinin were positive in the luteinized component whereas β-HCG and Ki-67 were positive in the multinucleate cell component. The diagnostic rarity and therapeutic dilemma of such a rare mixed tumor within a single ovary has proven to be an exceptional case and an excellent investigative opportunity.

Tratamento de macroprolactinoma com cabergolina em adolescente / An adolescent patient with prolactinoma treated with cabergoline

OBJETIVO: Relatar um caso de macroprolactinoma diagnosticado em adolescente e tratado com cabergolina. DESCRIÇÃO DO CASO: Paciente do sexo feminino, 15 anos, iniciou quadro clínico aos dez anos, com cefaleia, obesidade e não havia apresentado menarca ao diagnóstico. A dosagem sérica inicial de prolactina foi de 2492ng/mL (valor de referência: 19-25ng/mL). A ressonância nuclear magnética de crânio evidenciou formação expansiva selar e suprasselar compatível com macroadenoma hipofisário sem compressão do quiasma óptico. Seis meses após o início do tratamento com cabergolina, houve queda significativa dos níveis séricos de prolactina para 87,7ng/mL, com redução de 76 por cento no tamanho da lesão e melhora sintomática. COMENTÁRIOS: A ocorrência dos prolactinomas é rara na infância e na adolescência, mas o seu comportamento pode ser mais agressivo. O diagnóstico precoce e o manejo adequado do caso são importantes para um bom prognóstico.

OBJECTIVE: To report a teenager with macroprolactinoma treated with cabergoline. CASE DESCRIPTION: A 15-year old female adolescent reported the onset of headaches and obesity since ten years old. At diagnosis amenorrhea was not present. The serum level of prolactin was initially 2492ng/mL (reference values: 19-25ng/mL). Magnetic resonance images of the skull revealed an expansive sellar and suprasellar pituitary macroadenoma without optical chiasmal compression. Six months after starting treatment with cabergoline, there was a significant decrease of prolactin levels to 87.7 ng/mL, with a 76 percent reduction in the lesion size and she reported improvement of the symptoms. COMMENTS: In childhood and adolescence, prolactinomas are rare, but when they occur the clinical course is more aggressive. Early diagnosis and proper management of the disease are important for a good prognosis.

Amenorrea hipotalámica: consideraciones etiológicas y clínicas a raíz de una paciente / Hypothalamic amenorrhea: report of one case

Hypothalamic amenorrhea is secondary to the dysfunction of the hypothalamic pacemaker that regulates the pulsatile secretion of gonadotropin releasing hormone (GnRH). We report a 21 years old female with an isolated and persistent hypogonadotropic hypogonadism probably of congenital origin. The patient had a good olfactory function, did not have anatomical alterations and two GnRH stimulation tests showed a pre puberal pattern. Hormone replacement therapy allowed her to complete her puberal development.

Etiopatogenia de la amenorrea hipotalámica funcional: Interacción de las respuestas hormonales del sistema nervioso central y neuropéptidos periféricos / Ethiopatogeniae of functional hypothalamic amenorrhea: Interactions of central nervous system and peripherical neuropeptides responses

La amenorrea hipotalámica funcional (AHF) es una patología compleja provocada generalmente por el estrés psicológico, o por alteraciones psicógenas como la depresión en otras entidades como la amenorrea nerviosa y bulimia o en la excesiva actividad física (atletas competitivas, bailarinas clásicas) entre las causas más frecuentes. Habitualmente, estos factores se presentan asociados a dietas cuali y cuantitativamente inadecuadas, originando alteraciones en el balance energético (consumo /gasto calórico) y modificaciones en la composición corporal. En general, estas alteraciones se presentan combinadas e inducen reacciones hormonales tendientes a defender la homeostasis metabólica general. Los protagonistas de estos procesos adaptativos son un "circuito" constituido en el sistema nervioso central que se interrelaciona con otro "circuito periférico hormonal". Las manifestaciones del primero incluyen principalmente una atenuación funcional de los ejes hipotalamo-hipófiso-somatotrófico, prolactínico y tiroideo, amplificación de la secreción nocturna de la melatonina e hiperactividad adrenal. A su vez, las interrelaciones, a nivel hipotalámico, del eje CRH-ACTH-sistemas-opiodeo-dopaminérgico y la consiguiente inhibición de la secreción de Gn RH, constituye un mecanismo fisiopatológico crucial para el desarrollo de la disfunción gonadal. Por otro lado, el grado de alteración de la actividad pusátil del Gn RH está condicionado por la etiopatogenia, intensidad nosológica y el tiempo de evolución. La variabilidad y el tipo de irregularidades del ciclo menstrual, que frecuentemente son observados en las atletas competitivas, constituyen un reflejo representativo de ello. Por otro lado, el hipoestrogenismo e hipoandrogenismo de estas mujeres repercuten negativamente en el metabolismo general y osteocálcico, en particular. La osteopenia con su riesgo de fracturas, está omnipresente en este síndrome. No menos importante, el hipoestrogenismo es también uno de los factores de riesgo cardiovascular. No obstante, la administración de anticonceptivos que combinan estrógenos más progesterona, pueden aumentar significativamente los niveles de la proteína C reactiva, un reconocido y seguro marcador de riesgo cardiovascular. Ello induce a tomar precauciones en su utilización en estas amenorricas desnutridas. Asimismo, la administración de anticonceptivos no ha demostrado brindar un beneficio sustancial en el tratamiento de la osteosporosis de estas pacientes. El "circuito periférico", funcionalmente interrelacionado con el central, está principalmente compuesto por la leptina, adiponectina, ghrelin, insulina e IGF-1, péptidos provenientes del compartimiento graso ("adipocitokinas") tracto gastrointestinal superior, páncreas e hígado, respectivamente. Estos péptidos no sólo están involucrados en los mecanismos centrales del apetito y saciedad, sino que también participan en las respuestas de adaptación homeostática, tendientes a revertir los desvíos del metabolismo intermedio y fosfocálcico, que en grado variable afectan a estas pacientes. Conclusiones: la AHF relacionada en general con el estrés psicofísico prolongado y la mala nutrición, es un síndrome complejo, cuyas respuestas hormonales centrales y periféricas de adaptación, resultan primordiales en estas mujeres que padecen un cierto grado de compromiso nutritivometabólico general. Enfocando globalmente este abigarrado síndrome, estimamos que la AHF es, obviamente, un epifenómeno menos vital.

Functional Hypothalamic Amenorrhea (FHA) is a complex pathology produced by psicological stress, as observed in amenorrhea nervosa and bulimia or in intensive physical activity (athletas, competitions, classic dancing). Frequently, these factors are associated to quali-quantitative inadequated diets and therefore they induce alterations in the energetic balance (caloric intake, caloric expend and modification in body composition). These factors are usually combinated and they induce hormonal reactions trend to sustain the general metabolic homeostasis. The protagonists of this adaptative process are a "central nervous system circuit" interrelated with "periferical hormonal circuit". The responses to the first, mainly includes functional atenuation of Hypothalamic- Hipophyseal Somatotropic, Prolactin and Thyroid axes, amplification of nocturnal melatonine secretion and hyper adrenal activity. The relationships, to hypothalamic level, of CRH-ACTH, opioid, and dopaminergic systems, and the subsequent inhibition of Gn-RH secretion, constitute a physiopathological mechanism for the development of gonadal disfunction. On the other hand, the degree of alteration in the Gn-RH pulsatility is conditioned by the ethipahogenic, nosologic intensity and time of evolution. Variability and type of irregularity of menstrual cycle observed in competitive athletes, is a demonstrative example. On the other hand, hypoestrogenism and hypoandrogenism in these women affect negatively the general metabolism and, particularly, the osteocalcic system. Osteopenia with its fracture risk is omnipresence in this syndrome. As important as that hypoestrogenism is also one of cardiovascular factor risk. However the administration of contraceptives which combines estrogens plus progesterone, may significantly increase the C-reactive protein level, a known and safety marker of cardiovascular risk. Therefore it induces to take precautions in its administration in these unnourished amenorrheic women. Also, the administration of contraceptives has not showed a substantial benefit in osteoporosis treatment of this patients. A "periferic circuit", functionally interrelated with the "central circuit", is mainly composed by leptin, adiponectine, ghrelin, insulin and IGF1 peptides, coming from fat compartiment (adipokines), gastro-intestinal tract, pancreas and liver, respectively. These peptides, are not only involved in the central mechanisms of appetite and saciety, but they also participate in homeostatic adaptative responses, with the aim of to balance the alteration of middle and phosphocalcic metabolism which affect these patients in a variable form. CONCLUSIONS: FHA, generally related with psychic/physic stress and malnutrition, is a complex syndrome in which the central and peripheral adaptative hormonal responses are mainly important in these women which are affected of a kind of nutritive and general metabolic compromise. Analazing this complex process we considerate that the hypothalamic amenorrhea is obviously a less vital phenomenon.

Compromiso endocrinológico en el síndrome de POEMS: revisión a propósito de un caso / Secondary amenorrhea and hypothyroidism in a women with POEMS syndrome: report of one case

POEMS is an acronym for a syndrome characterized by osteosclerotic myeloma: polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes. Hypothyroidism, hypogonadism, diabetes mellitus and adrenal failure can be associated to POEMS syndrome. We report a 39 years old women with monoclonal gammopathy and paraparesis caused by a polyneuropathy, in whom a POEMS syndrome was diagnosed and treated with dexamethasone. The patient had a secondary amenorrhea and a clinical hypothyroidism with negative antithyroidal antibodies. Substitutionwith levothyroxine was started.

[Primary amenorrhea: An existing data at Ayat-allah Taleghani hospital [2003-5]]

Primary amenorrhea is one of the most important complaint of women in reproductive age. To determine the causes responsible for primary amenorrhea this study was performed on the basis of clinical, sonography and laboratories investigations. This case series study was performed at Ayat-allah Taleghani teaching hospital during the years of 2003-5. Data were collected from the patients who attended or referred to the clinics of gynecology or endocrinology wards since 10 years ago. 53 cases were evaluated. Mean age of the patients was 26.82 +/- 7.24 years when they were visited at the clinics. The most common cause of primary infertility was mullerian dysgenesis [n=19] according to clinical, sonography and laboratories investigations. Hypogonadotropic hypogonadism and congenital adrenal hyperplasia were seen in 12 and 8 patients, respectively. 5 patients had gonadal dysgenesis. This study suggest that registration of patients with primary amenorrhea in research center, can be beneficial for diagnosis and intervention

Ultrasonographic findings in a group of Iranian patients with primary amenorrhea

This study was performed to report the ultrasonographic finding and final diagnosis of a group of primary amenorrhea patients. Pelvic ultrasonography [US] was employed as the first diagnostic modality to evaluate primary amenorrhea in 53 patients who were admitted to gynecology or endocrinology clinics at Taleghani hospital from 2002 to 2006. US was based upon the presence or absence of the uterus and ovaries and any other abnormal sonographic findings. Karyotype analysis was also performed for all the patients. The uterus was not visualized in 16 [30%] patients: due to mllerian agenesis in 14 and testicular feminization and true hermaphroditism in two other patients. Mllerian anomalies with hematometrocolpos or hematometra were seen in 5 [9%] patients. Thirty-two [60%] patients had a normal or hypoplastic uterus. Pelvic US showed that ovaries were in normal limits in 39 [73%] patients; they were not visible in 9 patients. The report of pelvic US was not conclusive in 3 patients; 2 had an ovarian tumor or cyst. Irrespective of the presence or absence of the uterus, all patients with visible ovaries [except one] had a normal karyotype. US of the pelvis can be the initial diagnostic modality. Based on US findings, we can make decision for further work ups; there is no need to perform all paraclinical investigations for each patient

Treatment outcome of hyperprolactinaemic infertility with carbegoline in sub saharan Africa

Hyperprolactinaemia is a known cause of infertility. We explored the efficacy of carbegoline, the long acting dopamine agonist that was recently introduced into our medical practice. Seventy six patients with infertility secondary to hyperprolactinaemia were studied over a period of 20 weeks each. All the patients had carbegoline twice weekly for eight weeks. Two dosage regimen were used based on the pretreatment prolactin level; less than 50ng/ml had 0.25mg twice weekly [n=58] and 50ng/ml and above 0.5mg twice weekly [n=18]. Normalization of prolactin level was achieved in 75 [98.7%] patients. At the end of the study period, there was resumption of menstrual flow in 10 [76.9%] of the 13 patients that were amenorrhoiec and all the 39 [100%] patients that were oligomenorrhoeic had their normal menstrual cycle restored. Resumption of ovulatory cycles occurred in 87.7% of those with anovulatory cycles. Of the 76 patients, 69 [90.8%] got pregnant during the 20 weeks study. However, out of the 69 that got pregnant, 13 [18.8%] got pregnant while on carbegoline therapy. There was no case of carbegoline resistance or discontinuation recorded in this study. Carbegoline is a cost effective first line therapy in the management of infertile women with hyperprolactinaemia

Falência ovariana prematura: aspectos atuais / Premature ovarian failure: present aspects

A falência ovariana prematura (FOP) acomete aproximadamente 1:1000 mulheres antes dos 30 anos, 1:250 em torno dos 35 anos e de 1:100 aos 40 anos. Manifesta-se como amenorréia primária ou amenorréia secundária, não podendo ser considerada definitiva em todas as pacientes, uma vez que a concepção espontânea pode ocorrer em até 5-10 por cento das FOP. Na maioria dos casos apresenta-se na forma esporádica, pois apenas 5 por cento apresentam história familial. Entre as causas conhecidas estão as alterações cromossômicas, dos genes ligados ao cromossomo X e cromossomos autossômicos, doenças autoimunes, alterações tóxicas e iatrogênicas. Com relativa freqüência, a causa etiológica não é obtida, sendo então denominada de idiopática. O diagnóstico da FOP é feito baseado na história clínica e níveis elevados do hormônio folículo estimulante (FSH), sendo posteriormente investigadas as causas mais específicas. O manejo clínico visa o suporte emocional, o tratamento hormonal com estrogênios e progestogênios, a abordagem da infertilidade e a prevenção de co-morbidades como a osteoporose e potencial maior risco cardiovascular.

Premature ovarian failure occurs in approximately 1:1000 women before 30 years, 1:250 by 35 years and 1:100 by the age of 40. It is characterized by primary or secondary amenorrhea and cannot be considered as definitive because spontaneous conception may occur in 5 to 10 percent of cases. In 95 percent of cases, premature ovarian failure is sporadic. The known causes of premature ovarian failure include chromosomal defects, autoimmune diseases, exposure to radiation or chemotherapy, surgical procedures, and certain drugs. Frequently, however, the etiology is not clear and these cases are considered to be idiopathic. Premature ovarian failure is defined by gonadal failure and high serum follicle-stimulating hormone (FSH) levels. Clinical approach includes emotional support, hormonal therapy with estrogens and progesterone or progestogens, infertility treatment, and prevention of osteoporosis and potential cardiovascular risk.